Materiały źródłowe

• #1 Scleroderma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/scleroderma/diagnosis-treatment/drc-20351957

  There is no treatment that can cure or stop the overproduction of collagen that happens in scleroderma. But a variety of treatments can help control symptoms and prevent complications. […] Because scleroderma can affect so many different parts of the body, the choice of medicine varies depending on the symptoms. […] Medicines that suppress the immune system, such as those taken after organ transplants, may help reduce progression of some scleroderma symptoms, such as the thickening of the skin or worsening of lung damage. […] Physical or occupational therapists can help you improve your strength and mobility and maintain independence with daily tasks. […] Stem cell transplants might be an option for people who have serious symptoms that haven’t responded to more-common treatments. If the lungs or kidneys have been badly damaged, organ transplants might be considered.

• #2 Scleroderma: Symptoms, Causes & Treatment Options

  https://my.clevelandclinic.org/health/diseases/scleroderma

  Scleroderma is a chronic condition, which means you’ll need to manage your symptoms for a long time (maybe the rest of your life). […] There’s no cure for scleroderma, but your healthcare provider will help you find a combination of treatments that manages your symptoms and minimizes how much they impact your daily routine. […] Some common scleroderma treatments include: Skin treatments: You might need creams and moisturizers to prevent your skin from drying out. Immunosuppressants: Immunosuppressants stop your immune system from damaging healthy cells and tissues. Medicines to manage specific symptoms: For example, you might need medication to manage your blood pressure, improve your breathing, manage kidney failure or relieve gastrointestinal symptoms. Physical therapy: A physical therapist will help you improve how your body physically moves. Light therapy (phototherapy): Light therapy uses bright, focused UV light to treat skin conditions. It can help treat thickened skin. Stem cell transplants: Some people with severe symptoms might need a stem cell transplant. A stem cell transplant helps your body replace damaged blood cells with healthy donor cells. […] Cleveland Clinic takes a team approach to scleroderma treatment. We’ll help you slow its progression and manage your symptoms.

• #3 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Scleroderma can cause different symptoms in each person with the disease. Your treatment plan is designed just for your disease and individual symptoms. […] There are medications your rheumatologist can prescribe to help you manage localized scleroderma, usually skin symptoms and Raynauds phenomenon. […] Other medications treat systemic disease, or scleroderma that affects not just your skin, but your organs and connective tissues in your body. Systemic medications work to control your disease activity, or inflammation caused by your immune system, to help control serious conditions like interstitial lung disease (ILD) or pulmonary arterial hypertension (PAH). […] Medications for scleroderma may also treat symptoms that affect your quality of life and independence, like pain or stiffness in your joints or muscles, or GI problems like heartburn or acid reflux (GERD).

• #4 Scleroderma

  https://www.nhs.uk/conditions/scleroderma/

  Although there’s no cure for scleroderma, symptoms can usually be managed by a range of different treatments. […] The aim of treatment is to relieve symptoms, prevent the condition getting worse, detect and treat any complications (such as pulmonary hypertension) and help you maintain the use of affected parts of the body. […] Common treatments include: medication to improve circulation, medicines that reduce the activity of the immune system and slow the progression of the condition, steroids to relieve joint and muscle problems, moisturising affected areas of skin to help keep it supple and relieve itchiness, various medicines to control other symptoms (such as pain, heartburn and high blood pressure). […] If your symptoms are severe, surgery may be needed. For example, hard lumps under the skin may need to be removed, and tightened muscles may need to be loosened.

• #5 Scleroderma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952

  While there is no cure for scleroderma, treatments can ease symptoms, slow progression and improve quality of life. […] There are medicines that may help slow the progression of this lung damage. […] Prompt treatment of this condition is important to preserve kidney function.

• #6 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  No definitive treatment or universally accepted disease-modifying agent can alter the natural course of the disease. However, managing the affected system or systems has proven effective. Early diagnosis is crucial for achieving improved outcomes. Clinical evaluation and identification of affected organs and disease progression are critical for treatment efficacy. Furthermore, treatment goals must be holistic and tailored to optimize the quality of life for affected patients while also preventing further organ damage. Patient education about the disease and encouragement to engage in regular exercise, maintain a healthy diet and lifestyle, and seek emotional support should be considered for every individual with systemic sclerosis. […] Several agents have been investigated for different manifestations of systemic sclerosis, yet a generalized lack of large randomized controlled trials undermines their efficacy. Cyclophosphamide (for lung disease and skin disease), mycophenolate mofetil (for lung disease and skin disease), methotrexate (MTX; for skin disease, inflammatory arthritis, and myositis), azathioprine (for skin disease, lung disease, and myositis), and hydroxychloroquine (HCQ; for skin disease) stand out as some of the most frequently utilized immunosuppressive agents.

• #7 Scleroderma Treatment | UVA Health

  https://uvahealth.com/services/autoimmune-rheumatology/scleroderma

  Scleroderma is a rare disease of the connective tissue. While there’s no cure for this autoimmune disorder, experts at UVA Health can design a treatment plan to help relieve your symptoms. At UVA Health, you’ll have a team of experts to design a treatment plan. You’ll get care tailored based on your type of scleroderma and what part of your body it impacts, including: […] Physical therapy and exercise are important to maintain circulation, joint flexibility, and muscle strength.

• #8 Scleroderma Treatment Options : Johns Hopkins Scleroderma Center

  https://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/

  Because no two cases of Scleroderma are alike, identifying your disease subtype, stage, and involved organs is very important in determining the best course of action for treatment. Current therapies use medications that focus on the four main features of the disease: inflammation, autoimmunity, vascular disease, and tissue fibrosis. Your physician will work with you to identify the treatments that are best for you, but here are some common treatment options: […] The most popular approach to controlling the inflammatory phase of scleroderma is the use of immunosuppressive therapy. […] A major area of current research is the use of aggressive immunosuppressive therapy either with very-high-dose cyclophosphamide or with autologous bone marrow transplantation. […] Therefore, treatment of the vascular disease is now considered crucial to controlling the disease as a whole as well as preventing specific organ damage.

• #9 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  New trials and increased understanding of pathogenesis have produced a number of promising therapies for SSc-related skin disease. […] Methotrexate is the most commonly administered immunosuppressive in patients with SSc and has been shown to be effective in multiple prospective trials. […] The use of MMF in treating SSc is supported by several small observational studies. […] Intravenous immunoglobulin (IVIG) is used to treat a host of autoimmune diseases and its use in patients with SSc was first reported in 2000 in three patients with rapidly progressing skin disease. […] Rituximab is a chimeric monoclonal antibody that targets CD20, a B-lymphocyte surface molecule that assists in differentiation and development of B cells into plasma cells. […] UVA-1 (340-400 nm) phototherapy has been used in various T cell-mediated diseases, such as atopic dermatitis and mycosis fungoides, as it is thought to penetrate deep into the dermis to induce apoptosis of infiltrating T cells. […] The use of tocilizumab, an interleukin 6 receptor alpha inhibitor, has previously been studied, and phase III studies are under way. […] As such, abatacept, a medication that interferes with the activation of T cells, is thought to be a promising therapeutic for the treatment of SSc.

• #10 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs and has significant clinical sequelae. Management of SSc cutaneous disease remains challenging and often is driven by extracutaneous manifestations. Methotrexate is the typical first-line therapy for patients with early progressive cutaneous disease. […] However, in patients with diffuse progressive skin disease and inflammatory arthritis, methotrexate or rituximab monotherapy should be considered. First-line therapy for patients with concomitant myositis includes methotrexate or intravenous immunoglobulin (IVIG). For patients with both cutaneous findings and interstitial lung disease, studies have suggested the efficacy of mycophenolate mofetil or rituximab. Second-line therapies, including UVA-1 phototherapy, IVIG, or rituximab, can be considered in patients with disease refractory to first-line treatments. […] Clinical trials investigating the utility of emerging therapies such as abatacept and tocilizumab in the treatment of SSc are under way, and preliminary results are promising. […] Overall, SSc is a clinically heterogenous disease that affects multiple organ systems. Providers should assess extracutaneous involvement and use evidence-based recommendations to select the most appropriate therapy for patients with SSc.

• #11 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Opioids are also called narcotics. They include prescription medications used to relieve pain only. […] Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. […] Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. […] Cyclophosphamide is another immunosuppressant medication thats been shown to improve skin fibrosis in scleroderma. […] Methotrexate is another immunosuppressant thats been used for decades to treat inflammatory diseases, including rheumatoid arthritis. […] Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. […] People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux.

• #12 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Opioids are also called narcotics. They include prescription medications used to relieve pain only. […] Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. […] Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. […] Cyclophosphamide is another immunosuppressant medication thats been shown to improve skin fibrosis in scleroderma. […] Methotrexate is another immunosuppressant thats been used for decades to treat inflammatory diseases, including rheumatoid arthritis. […] Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. […] People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux.

• #13 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  New trials and increased understanding of pathogenesis have produced a number of promising therapies for SSc-related skin disease. […] Methotrexate is the most commonly administered immunosuppressive in patients with SSc and has been shown to be effective in multiple prospective trials. […] The use of MMF in treating SSc is supported by several small observational studies. […] Intravenous immunoglobulin (IVIG) is used to treat a host of autoimmune diseases and its use in patients with SSc was first reported in 2000 in three patients with rapidly progressing skin disease. […] Rituximab is a chimeric monoclonal antibody that targets CD20, a B-lymphocyte surface molecule that assists in differentiation and development of B cells into plasma cells. […] UVA-1 (340-400 nm) phototherapy has been used in various T cell-mediated diseases, such as atopic dermatitis and mycosis fungoides, as it is thought to penetrate deep into the dermis to induce apoptosis of infiltrating T cells. […] The use of tocilizumab, an interleukin 6 receptor alpha inhibitor, has previously been studied, and phase III studies are under way. […] As such, abatacept, a medication that interferes with the activation of T cells, is thought to be a promising therapeutic for the treatment of SSc.

• #14 State-of-the-art evidence in the treatment of systemic sclerosis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00909-5

  Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous SSc (dcSSc) and the use of organ-specific therapies, has improved, as evident from randomized clinical trials. Treatments for early dcSSc include immunosuppressive agents such as mycophenolate mofetil, methotrexate, cyclophosphamide, rituximab and tocilizumab. Patients with rapidly progressive early dcSSc might be eligible for autologous haematopoietic stem cell transplantation, which can improve survival. […] Morbidity from interstitial lung disease and pulmonary arterial hypertension is improving with the use of proven therapies. Mycophenolate mofetil has surpassed cyclophosphamide as the initial treatment for SSc-interstitial lung disease. Nintedanib and possibly perfinidone can be considered in SSc pulmonary fibrosis. Pulmonary arterial hypertension is frequently treated with initial combination therapy (for example, with phosphodiesterase 5 inhibitors and endothelin receptor antagonists) and, if necessary, the addition of a prostacyclin analogue. Raynaud phenomenon and digital ulcers are treated with dihydropyridine calcium channel blockers (especially nifedipine), then phosphodiesterase 5 inhibitors or intravenous iloprost. Bosentan can reduce the development of new digital ulcers.

• #15 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  Mycophenolate mofetil (with a target dose of 1.5-3 g daily in 2 divided doses) has shown benefits in managing systemic sclerosis-related interstitial lung disease. […] When no contraindication exists, supplemental oxygen, diuretics, and anticoagulation are recommended, alongside patient education, a healthy lifestyle, and exercise as tolerated. […] ACE inhibitors represent the sole effective treatment for scleroderma renal crisis. Initiation should occur upon the earliest signs, with a maximum tolerated dose utilized.

• #16 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Opioids are also called narcotics. They include prescription medications used to relieve pain only. […] Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. […] Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. […] Cyclophosphamide is another immunosuppressant medication thats been shown to improve skin fibrosis in scleroderma. […] Methotrexate is another immunosuppressant thats been used for decades to treat inflammatory diseases, including rheumatoid arthritis. […] Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. […] People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux.

• #17 Scleroderma Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/scleroderma.html

  There is currently no single treatment for scleroderma that is reliably effective. Treatment choices depend on the organ involved, other medications and medical problems, and patient preference. […] A host of drugs are commonly prescribed and may have benefit. Among the most commonly prescribed drugs for scleroderma are: […] Mycophenolate mofetil (Cellcept) and mycophenolate sodium (Myfortic) may be especially helpful for people with skin or lung involvement. […] Methotrexate (Rheumatrex, Trexall, others) may be particularly helpful for skin involvement, arthritis, or myositis. […] Cyclophosphamide (Cytoxan, Neosar) decreases the activity of the immune system and has been shown to improve lung function when used along with corticosteroids in people with inflammation in the lungs. […] Glucocorticoids can be used to relieve inflammation of the membrane surrounding the heart (pericarditis), arthritis, and inflammation of the lungs (pneumonitis) or muscles (myositis).

• #18 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #19 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Opioids are also called narcotics. They include prescription medications used to relieve pain only. […] Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. […] Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. […] Cyclophosphamide is another immunosuppressant medication thats been shown to improve skin fibrosis in scleroderma. […] Methotrexate is another immunosuppressant thats been used for decades to treat inflammatory diseases, including rheumatoid arthritis. […] Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. […] People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux.

• #20 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  New trials and increased understanding of pathogenesis have produced a number of promising therapies for SSc-related skin disease. […] Methotrexate is the most commonly administered immunosuppressive in patients with SSc and has been shown to be effective in multiple prospective trials. […] The use of MMF in treating SSc is supported by several small observational studies. […] Intravenous immunoglobulin (IVIG) is used to treat a host of autoimmune diseases and its use in patients with SSc was first reported in 2000 in three patients with rapidly progressing skin disease. […] Rituximab is a chimeric monoclonal antibody that targets CD20, a B-lymphocyte surface molecule that assists in differentiation and development of B cells into plasma cells. […] UVA-1 (340-400 nm) phototherapy has been used in various T cell-mediated diseases, such as atopic dermatitis and mycosis fungoides, as it is thought to penetrate deep into the dermis to induce apoptosis of infiltrating T cells. […] The use of tocilizumab, an interleukin 6 receptor alpha inhibitor, has previously been studied, and phase III studies are under way. […] As such, abatacept, a medication that interferes with the activation of T cells, is thought to be a promising therapeutic for the treatment of SSc.

• #21 Systemic sclerosis

  https://dermnetnz.org/topics/systemic-sclerosis

  Abatacept (anti-cytotoxic T-cell lymphocyte-associated inhibitor [CTLA] 4 therapy shows a possible trend towards skin improvement in inflammatory disease. […] Autologous stem cell transplant for severe and acute progressive disease in selected patients seen within specialist centres. […] Intravenous immunoglobulin may be useful for skin and gastrointestinal tract. […] Tofacitinib well-tolerated, prevents bleomycin-induced skin fibrosis in a mouse model; a small study showed no clinical improvement in skin sclerosis, but larger studies are needed. […] Endothelin 1 antagonists (bosentan), Phosphodiesterase-5 inhibitors (tadalafil), Guanylate cyclase agonists (riociguat), Prostacyclin agonists (epoprostenol; selexipag) are used for vasodilation. […] General measures such as keeping warm, stretching exercises for joints to reduce the risk of worsening contractures and microstomia, and specifically-related physiotherapy can all be beneficial.

• #22 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  New trials and increased understanding of pathogenesis have produced a number of promising therapies for SSc-related skin disease. […] Methotrexate is the most commonly administered immunosuppressive in patients with SSc and has been shown to be effective in multiple prospective trials. […] The use of MMF in treating SSc is supported by several small observational studies. […] Intravenous immunoglobulin (IVIG) is used to treat a host of autoimmune diseases and its use in patients with SSc was first reported in 2000 in three patients with rapidly progressing skin disease. […] Rituximab is a chimeric monoclonal antibody that targets CD20, a B-lymphocyte surface molecule that assists in differentiation and development of B cells into plasma cells. […] UVA-1 (340-400 nm) phototherapy has been used in various T cell-mediated diseases, such as atopic dermatitis and mycosis fungoides, as it is thought to penetrate deep into the dermis to induce apoptosis of infiltrating T cells. […] The use of tocilizumab, an interleukin 6 receptor alpha inhibitor, has previously been studied, and phase III studies are under way. […] As such, abatacept, a medication that interferes with the activation of T cells, is thought to be a promising therapeutic for the treatment of SSc.

• #23 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  In patients with systemic sclerosis-related interstitial lung disease, a clinically meaningful decline of 10% or more in lung function was seen in 24.5% of placebo recipients, compared with 8.6% of tocilizumab recipients. […] Other agents are currently being studied for skin and lung involvement. For example, trials of rituximab have yielded promising results, with improvement of skin fibrosis and prevention of worsening lung fibrosis. […] The tyrosine kinase inhibitor nintedanib is approved for slowing the rate of decline in pulmonary function in adults with systemic sclerosis-related interstitial lung disease. […] Phototherapy using longer-wavelength ultraviolet A (UVA) light (ie, UVA1, 340-400 nm) has proved beneficial for cutaneous lesions in scleroderma. […] Hematopoietic stem cell transplantation (HSCT) has been shown to be effective. However, it is associated with a high rate of procedure-related complications.

• #24 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  The authors recommend using an evidence-based algorithm when considering therapeutic options for patients with SSc. […] Importantly, patients with early-onset diffuse skin manifestations respond best to treatment. […] In patients with skin disease and ILD, studies have suggested that MMF or rituximab may have the best efficacy. […] Lastly, in patients with solely cutaneous manifestations, treatment should be dictated by disease severity as measured by mRSSs after careful assessment for internal organ involvement. […] SSc is a connective tissue disease that leads to progressive fibrosis of the skin and internal organs. Treatment of scleroderma continues to be a challenge as targeted therapies are lacking. […] Although methotrexate remains the most commonly used therapy in treatment of scleroderma-related cutaneous disease, studies investigating the utility and efficacy of other therapeutics, including MMF, IVIG, and UVA-1 phototherapy, are promising. […] When treating scleroderma, clinicians should consider the patients specific organ complications to guide therapeutic selection and management.

• #25 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. […] Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. […] Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. […] Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. […] Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. […] People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. […] Pulmonary hypertension medications include: Ambrisentan, bosentan and macitentan, Sildenafil and tadalafil, Epoprostenol, iloprost, tresprostinil, Selexipag and riociguat. […] Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital.

• #26 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  Current treatment of systemic sclerosis is directed toward managing complications and providing symptomatic relief. In addition, a range of disease-modifying treatments have been investigated. […] Disease-modifying treatment aims at inhibiting tissue fibrosis and vascular and immune system alterations, which are the three crucial components of disease pathogenesis. […] Therapies targeting cytokine signalling, including interleukin-6 (IL-6), and other immune-inflammatory therapies, have produced promising results. […] To date, the US Food and Drug Administration (FDA) has approved nintedanib and as well as tocilizumab for refractory, progressive interstitial lung disease due to systemic sclerosis. […] A phase 3 trial of tocilizumab in systemic sclerosis failed to meet its primary endpoint of improvement in modified Rodnan skin score; however, tocilizumab therapy did result in preservation of lung function.

• #27 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  Current treatment of systemic sclerosis is directed toward managing complications and providing symptomatic relief. In addition, a range of disease-modifying treatments have been investigated. […] Disease-modifying treatment aims at inhibiting tissue fibrosis and vascular and immune system alterations, which are the three crucial components of disease pathogenesis. […] Therapies targeting cytokine signalling, including interleukin-6 (IL-6), and other immune-inflammatory therapies, have produced promising results. […] To date, the US Food and Drug Administration (FDA) has approved nintedanib and as well as tocilizumab for refractory, progressive interstitial lung disease due to systemic sclerosis. […] A phase 3 trial of tocilizumab in systemic sclerosis failed to meet its primary endpoint of improvement in modified Rodnan skin score; however, tocilizumab therapy did result in preservation of lung function.

• #28 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  In the prospective Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial, a phase 3 comparison of autologous HSCT with 12 successive monthly intravenous pulses of cyclophosphamide in 156 patients with early diffuse cutaneous systemic sclerosis, HCST was associated with higher treatment-related mortality than in the first year after treatment. […] Numerous experimental drugs or interventions have been investigated for treatment of skin induration and fibrosis in systemic sclerosis. Interventions that have demonstrated benefit include the following: D-penicillamine, Bovine collagen, Methotrexate, Mycophenolate mofetil, Allogeneic bone marrow transplantation. […] Interventions that have failed to demonstrate significant benefit for treatment of skin induration and fibrosis in systemic sclerosis include the following: Human relaxin, Interferon-alpha, Antitransforming growth factor beta antibodies.

• #29 Systemic sclerosis

  https://dermnetnz.org/topics/systemic-sclerosis

  Abatacept (anti-cytotoxic T-cell lymphocyte-associated inhibitor [CTLA] 4 therapy shows a possible trend towards skin improvement in inflammatory disease. […] Autologous stem cell transplant for severe and acute progressive disease in selected patients seen within specialist centres. […] Intravenous immunoglobulin may be useful for skin and gastrointestinal tract. […] Tofacitinib well-tolerated, prevents bleomycin-induced skin fibrosis in a mouse model; a small study showed no clinical improvement in skin sclerosis, but larger studies are needed. […] Endothelin 1 antagonists (bosentan), Phosphodiesterase-5 inhibitors (tadalafil), Guanylate cyclase agonists (riociguat), Prostacyclin agonists (epoprostenol; selexipag) are used for vasodilation. […] General measures such as keeping warm, stretching exercises for joints to reduce the risk of worsening contractures and microstomia, and specifically-related physiotherapy can all be beneficial.

• #30 Scleroderma Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/scleroderma.html

  There is currently no single treatment for scleroderma that is reliably effective. Treatment choices depend on the organ involved, other medications and medical problems, and patient preference. […] A host of drugs are commonly prescribed and may have benefit. Among the most commonly prescribed drugs for scleroderma are: […] Mycophenolate mofetil (Cellcept) and mycophenolate sodium (Myfortic) may be especially helpful for people with skin or lung involvement. […] Methotrexate (Rheumatrex, Trexall, others) may be particularly helpful for skin involvement, arthritis, or myositis. […] Cyclophosphamide (Cytoxan, Neosar) decreases the activity of the immune system and has been shown to improve lung function when used along with corticosteroids in people with inflammation in the lungs. […] Glucocorticoids can be used to relieve inflammation of the membrane surrounding the heart (pericarditis), arthritis, and inflammation of the lungs (pneumonitis) or muscles (myositis).

• #31 Scleroderma Treatment Options : Johns Hopkins Scleroderma Center

  https://www.hopkinsscleroderma.org/patients/scleroderma-treatment-options/

  Because no two cases of Scleroderma are alike, identifying your disease subtype, stage, and involved organs is very important in determining the best course of action for treatment. Current therapies use medications that focus on the four main features of the disease: inflammation, autoimmunity, vascular disease, and tissue fibrosis. Your physician will work with you to identify the treatments that are best for you, but here are some common treatment options: […] The most popular approach to controlling the inflammatory phase of scleroderma is the use of immunosuppressive therapy. […] A major area of current research is the use of aggressive immunosuppressive therapy either with very-high-dose cyclophosphamide or with autologous bone marrow transplantation. […] Therefore, treatment of the vascular disease is now considered crucial to controlling the disease as a whole as well as preventing specific organ damage.

• #32 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  Specific Therapies in Scleroderma […] Raynaud phenomenon treatment aims to prevent digital ischemia and ulcers, which are easier to prevent than treat. Conservative management for the Raynaud phenomenon is still the cornerstone of management, and patients are advised to keep their extremities and body warm, avoid smoking and stress, and eliminate sympathomimetic medications when possible. […] Vasodilator therapy has demonstrated efficacy in Raynaud syndrome. Dihydropyridine calcium channel blockers, such as nifedipine (30-120 mg/d) or amlodipine (5-20 mg/d), are the first-line agents. […] Several immunosuppressive agents have been used in sclerodactyly, including MTX, HCQ, mycophenolate mofetil, and cyclophosphamide. […] IV methylprednisolone is typically discontinued after 3 to 4 months of symptom resolution. Oral prednisone is tapered slowly over 3 to 4 months.

• #33 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  Specific Therapies in Scleroderma […] Raynaud phenomenon treatment aims to prevent digital ischemia and ulcers, which are easier to prevent than treat. Conservative management for the Raynaud phenomenon is still the cornerstone of management, and patients are advised to keep their extremities and body warm, avoid smoking and stress, and eliminate sympathomimetic medications when possible. […] Vasodilator therapy has demonstrated efficacy in Raynaud syndrome. Dihydropyridine calcium channel blockers, such as nifedipine (30-120 mg/d) or amlodipine (5-20 mg/d), are the first-line agents. […] Several immunosuppressive agents have been used in sclerodactyly, including MTX, HCQ, mycophenolate mofetil, and cyclophosphamide. […] IV methylprednisolone is typically discontinued after 3 to 4 months of symptom resolution. Oral prednisone is tapered slowly over 3 to 4 months.

• #34 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  Specific Therapies in Scleroderma […] Raynaud phenomenon treatment aims to prevent digital ischemia and ulcers, which are easier to prevent than treat. Conservative management for the Raynaud phenomenon is still the cornerstone of management, and patients are advised to keep their extremities and body warm, avoid smoking and stress, and eliminate sympathomimetic medications when possible. […] Vasodilator therapy has demonstrated efficacy in Raynaud syndrome. Dihydropyridine calcium channel blockers, such as nifedipine (30-120 mg/d) or amlodipine (5-20 mg/d), are the first-line agents. […] Several immunosuppressive agents have been used in sclerodactyly, including MTX, HCQ, mycophenolate mofetil, and cyclophosphamide. […] IV methylprednisolone is typically discontinued after 3 to 4 months of symptom resolution. Oral prednisone is tapered slowly over 3 to 4 months.

• #35 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  The management of patients with SSc includes non-pharmacological and pharmacological interventions. […] In 2009, EULAR and European Scleroderma Trial and Research (EUSTAR) working group developed evidence-based, consensus-derived recommendations for the pharmacological management of SSc. […] The process described above lasted from February 2022 to May 2023 and resulted in 22 recommendations, compared with 16 in 2017. […] The recommendations with grade and task force level of agreement are described in table 1. […] The evidence informing this set of recommendations focuses on the same classes of drugs for the management of RP, DU disease and pulmonary artery hypertension (PAH) in SSc. […] Dihydropyridine-type calcium antagonists, usually oral nifedipine, should be used as first-line therapy for SSc-RP.

• #36 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #37 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Blood pressure medicine is prescribed to manage Raynauds phenomenon. These medications open constricted blood vessels. […] Phosphodiesterase-5 (PED-5) inhibitors are medications that can be used to treat more severe Raynauds phenomenon symptoms, such as ulcerations on the fingers. […] Topical nitroglycerine cream or paste can be used to treat mild, localized Raynauds symptoms like a skin ulcer. […] Pain is a common symptom for people living with scleroderma. You may have pain, stiffness or aches in your joints or muscles. Medications can help you manage pain, but they may have other side effects that could be serious. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes called anti-inflammatories. These are common pain medications. […] Steroids, also called glucocorticoids or corticosteroids, are stronger medications to treat flare-ups of inflammation that cause pain or joint swelling.

• #38 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #39 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Blood pressure medicine is prescribed to manage Raynauds phenomenon. These medications open constricted blood vessels. […] Phosphodiesterase-5 (PED-5) inhibitors are medications that can be used to treat more severe Raynauds phenomenon symptoms, such as ulcerations on the fingers. […] Topical nitroglycerine cream or paste can be used to treat mild, localized Raynauds symptoms like a skin ulcer. […] Pain is a common symptom for people living with scleroderma. You may have pain, stiffness or aches in your joints or muscles. Medications can help you manage pain, but they may have other side effects that could be serious. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes called anti-inflammatories. These are common pain medications. […] Steroids, also called glucocorticoids or corticosteroids, are stronger medications to treat flare-ups of inflammation that cause pain or joint swelling.

• #40 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Blood pressure medicine is prescribed to manage Raynauds phenomenon. These medications open constricted blood vessels. […] Phosphodiesterase-5 (PED-5) inhibitors are medications that can be used to treat more severe Raynauds phenomenon symptoms, such as ulcerations on the fingers. […] Topical nitroglycerine cream or paste can be used to treat mild, localized Raynauds symptoms like a skin ulcer. […] Pain is a common symptom for people living with scleroderma. You may have pain, stiffness or aches in your joints or muscles. Medications can help you manage pain, but they may have other side effects that could be serious. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes called anti-inflammatories. These are common pain medications. […] Steroids, also called glucocorticoids or corticosteroids, are stronger medications to treat flare-ups of inflammation that cause pain or joint swelling.

• #41 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #42 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  The management of patients with SSc includes non-pharmacological and pharmacological interventions. […] In 2009, EULAR and European Scleroderma Trial and Research (EUSTAR) working group developed evidence-based, consensus-derived recommendations for the pharmacological management of SSc. […] The process described above lasted from February 2022 to May 2023 and resulted in 22 recommendations, compared with 16 in 2017. […] The recommendations with grade and task force level of agreement are described in table 1. […] The evidence informing this set of recommendations focuses on the same classes of drugs for the management of RP, DU disease and pulmonary artery hypertension (PAH) in SSc. […] Dihydropyridine-type calcium antagonists, usually oral nifedipine, should be used as first-line therapy for SSc-RP.

• #43 Scleroderma and Systemic Sclerosis (SSc): An Overview – HSS.edu

  https://www.hss.edu/conditions_scleroderma-and-systemic-sclerosis-overview.asp

  The first step in treatment of this issue is usually a calcium channel blocker (amlodipine or nifedipine). […] Wound care is also important in the treatment of ulcers. Various ointments may be prescribed, and if the ulcers become infected the physician will prescribe antibiotics. […] Interstitial lung disease can be treated with immunosuppressive therapies such as cyclophosphamide, Cellcept (mycophenolate mofetil), Actemra (tocilizumab), and/or anti-fibrotic treatments such as Ofev (nintedanib). […] Muscle disease in scleroderma can be noninflammatory or inflammatory. Inflammatory muscle disease can be treated with immunosuppressant medications (methotrexate, azathioprine, corticosteroids, rituximab) or IVIG (intravenous immune globulin). […] Individuals with systemic sclerosis can have inflammatory arthritis, which can respond to low dose steroids, hydroxychloroquine (Plaquenil), methotrexate, mycophenolate mofetil (Cellcept) or other therapies. […] Immunosuppressive agents like methotrexate and mycophenolate mofetil (Cellcept) are frequently used. […] Rehabilitation/Physical and occupational therapy (PT/OT): This is essential to overall health and maintenance function.

• #44 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  Treatment measures for pruritus include the following: Moisturizers, Histamine 1 (H1) and histamine 2 (H2) blockers, Tricyclic antidepressants, Trazodone. […] Raynaud phenomenon can be treated with the following agents: Calcium channel blockers, Prazosin, Prostaglandin derivatives, Dipyridamole, Aspirin, Topical nitrates. […] Sildenafil, an inhibitor of phosphodiesterase 5 (PDE-5), has been approved for treatment of pulmonary hypertension. […] In the event of thrombosis and vascular flow compromise, a tissue plasminogen activator, heparin, and urokinase may be necessary. […] Digital sympathectomy and botulinum toxin injections may be used in patients with severe Raynaud phenomenon who have an unrelenting acute attack and who are threatened by digital loss. […] There are no specific dietary recommendations in patients with systemic sclerosis.

• #45 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. […] Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. […] Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. […] Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. […] Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. […] People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. […] Pulmonary hypertension medications include: Ambrisentan, bosentan and macitentan, Sildenafil and tadalafil, Epoprostenol, iloprost, tresprostinil, Selexipag and riociguat. […] Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital.

• #46 State-of-the-art evidence in the treatment of systemic sclerosis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00909-5

  Systemic sclerosis (SSc) is a rare autoimmune connective tissue disease with multi-organ involvement, fibrosis and vasculopathy. Treatment in SSc, including early diffuse cutaneous SSc (dcSSc) and the use of organ-specific therapies, has improved, as evident from randomized clinical trials. Treatments for early dcSSc include immunosuppressive agents such as mycophenolate mofetil, methotrexate, cyclophosphamide, rituximab and tocilizumab. Patients with rapidly progressive early dcSSc might be eligible for autologous haematopoietic stem cell transplantation, which can improve survival. […] Morbidity from interstitial lung disease and pulmonary arterial hypertension is improving with the use of proven therapies. Mycophenolate mofetil has surpassed cyclophosphamide as the initial treatment for SSc-interstitial lung disease. Nintedanib and possibly perfinidone can be considered in SSc pulmonary fibrosis. Pulmonary arterial hypertension is frequently treated with initial combination therapy (for example, with phosphodiesterase 5 inhibitors and endothelin receptor antagonists) and, if necessary, the addition of a prostacyclin analogue. Raynaud phenomenon and digital ulcers are treated with dihydropyridine calcium channel blockers (especially nifedipine), then phosphodiesterase 5 inhibitors or intravenous iloprost. Bosentan can reduce the development of new digital ulcers.

• #47 Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2008/1015/p961.html

  In addition to therapies to control gastrointestinal symptoms and prevent GERD complications, patients with systemic sclerosis and gastric antral vascular ectasia may require endoscopic laser coagulation to decrease the risk of bleeding. […] Results from two recent randomized trials suggest that oral or intravenous cyclophosphamide is beneficial in patients with early and progressive interstitial lung disease. […] All patients with systemic sclerosis should be advised to check their blood pressure at home on a regular basis. Any persistent elevations should prompt a medical evaluation and treatment with ACE inhibitors if scleroderma renal crisis is suspected. ACE inhibitors should be used to control hypertension despite rising serum creatinine levels or the initiation of dialysis because they are essential for preserving and restoring renal function.

• #48 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  In patients with systemic sclerosis-related interstitial lung disease, a clinically meaningful decline of 10% or more in lung function was seen in 24.5% of placebo recipients, compared with 8.6% of tocilizumab recipients. […] Other agents are currently being studied for skin and lung involvement. For example, trials of rituximab have yielded promising results, with improvement of skin fibrosis and prevention of worsening lung fibrosis. […] The tyrosine kinase inhibitor nintedanib is approved for slowing the rate of decline in pulmonary function in adults with systemic sclerosis-related interstitial lung disease. […] Phototherapy using longer-wavelength ultraviolet A (UVA) light (ie, UVA1, 340-400 nm) has proved beneficial for cutaneous lesions in scleroderma. […] Hematopoietic stem cell transplantation (HSCT) has been shown to be effective. However, it is associated with a high rate of procedure-related complications.

• #49 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #50 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #51 Scleroderma

  https://rheumatology.org/patients/scleroderma

  While there is no cure for scleroderma, there are treatments to help manage symptoms and improve quality of life. […] For Raynauds, blood pressure medications called calcium channel blockers or PDE-5 inhibitors like sildenafil (Viagra) and tadalafil (Cialis), can improve circulation. Antacids and proton pump inhibitors (omeprazole) can help with heartburn. There are no approved medications for skin thickening, but methotrexate (Rheumatrex) and mycophenolate mofetil (Cellcept) can be used early in the disease depending on the presence of other symptoms such as joint, muscle, or lung disease. Mycophenolate is also used to treat patients who develop scarring or inflammation of the lungs (known as interstitial lung disease). In more severe lung cases, treatment with tocilizumab, which is a biologic medication might be helpful. In patients with even more progressive disease, treatment with nintedanib (Ofev) or rituximab can be tried. Other treatments for a serious lung complication (pulmonary arterial hypertension) can relax constricted blood vessels to ease high blood pressure. In some patients with diffuse forms of scleroderma a bone marrow transplant can also be considered.

• #52 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  Mycophenolate mofetil (with a target dose of 1.5-3 g daily in 2 divided doses) has shown benefits in managing systemic sclerosis-related interstitial lung disease. […] When no contraindication exists, supplemental oxygen, diuretics, and anticoagulation are recommended, alongside patient education, a healthy lifestyle, and exercise as tolerated. […] ACE inhibitors represent the sole effective treatment for scleroderma renal crisis. Initiation should occur upon the earliest signs, with a maximum tolerated dose utilized.

• #53 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. […] Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. […] Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. […] Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. […] Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. […] People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. […] Pulmonary hypertension medications include: Ambrisentan, bosentan and macitentan, Sildenafil and tadalafil, Epoprostenol, iloprost, tresprostinil, Selexipag and riociguat. […] Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital.

• #54 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #55 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #56 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. […] Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. […] Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. […] Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. […] Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. […] People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. […] Pulmonary hypertension medications include: Ambrisentan, bosentan and macitentan, Sildenafil and tadalafil, Epoprostenol, iloprost, tresprostinil, Selexipag and riociguat. […] Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital.

• #57 Scleroderma: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/scleroderma-treatment

  There is currently no cure for scleroderma. However, treatment can improve a person’s quality of life. […] Treatment works best when started early. If the diagnosis is scleroderma, treatment is most effective when started shortly after the disease has begun. At that time, physical and occupational therapy can help you keep your ability to straighten and bend your joints and maintain your daily life. […] Started early, treatments like phototherapy (light therapy) and medicines that work on the immune system like methotrexate and cyclosporine can help diminish scleroderma. […] Physical therapy can help you retain the ability to straighten and bend your joints. […] While there are many treatment options, the U.S. Food and Drug Administration (FDA) has not approved any drug to treat scleroderma on the skin. Still, there are effective treatments to treat the skin problems.

• #58 Scleroderma: Symptoms, Causes & Treatment Options

  https://my.clevelandclinic.org/health/diseases/scleroderma

  Scleroderma is a chronic condition, which means you’ll need to manage your symptoms for a long time (maybe the rest of your life). […] There’s no cure for scleroderma, but your healthcare provider will help you find a combination of treatments that manages your symptoms and minimizes how much they impact your daily routine. […] Some common scleroderma treatments include: Skin treatments: You might need creams and moisturizers to prevent your skin from drying out. Immunosuppressants: Immunosuppressants stop your immune system from damaging healthy cells and tissues. Medicines to manage specific symptoms: For example, you might need medication to manage your blood pressure, improve your breathing, manage kidney failure or relieve gastrointestinal symptoms. Physical therapy: A physical therapist will help you improve how your body physically moves. Light therapy (phototherapy): Light therapy uses bright, focused UV light to treat skin conditions. It can help treat thickened skin. Stem cell transplants: Some people with severe symptoms might need a stem cell transplant. A stem cell transplant helps your body replace damaged blood cells with healthy donor cells. […] Cleveland Clinic takes a team approach to scleroderma treatment. We’ll help you slow its progression and manage your symptoms.

• #59 Scleroderma > Fact Sheets > Yale Medicine

  https://www.yalemedicine.org/conditions/scleroderma

  Treatment includes medications, physical therapy, lifestyle changes, phototherapy, and surgery. […] Scleroderma treatments aim to alleviate symptoms or prevent further damage to skin and organs. The choice of treatment depends on a patients symptoms, the extent of the disease, and which organs are involved. […] Some medications used to treat people with scleroderma include: Topical medications (such as ointments or creams), including corticosteroids, tacrolimus (a drug that suppresses the immune system), and vitamin D analogs, may be used to treat skin lesions caused by scleroderma. […] Corticosteroids given orally, as injections, or intravenously help reduce inflammation, as well as joint and muscle pain. […] Immunosuppressants medications that suppress the immune system such as methotrexate, mycophenolate mofetil, and rituximab may be used to treat skin lesions and inflammation of internal organs.

• #60 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  Specific Therapies in Scleroderma […] Raynaud phenomenon treatment aims to prevent digital ischemia and ulcers, which are easier to prevent than treat. Conservative management for the Raynaud phenomenon is still the cornerstone of management, and patients are advised to keep their extremities and body warm, avoid smoking and stress, and eliminate sympathomimetic medications when possible. […] Vasodilator therapy has demonstrated efficacy in Raynaud syndrome. Dihydropyridine calcium channel blockers, such as nifedipine (30-120 mg/d) or amlodipine (5-20 mg/d), are the first-line agents. […] Several immunosuppressive agents have been used in sclerodactyly, including MTX, HCQ, mycophenolate mofetil, and cyclophosphamide. […] IV methylprednisolone is typically discontinued after 3 to 4 months of symptom resolution. Oral prednisone is tapered slowly over 3 to 4 months.

• #61 Scleroderma: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/scleroderma-treatment

  There is currently no cure for scleroderma. However, treatment can improve a person’s quality of life. […] Treatment works best when started early. If the diagnosis is scleroderma, treatment is most effective when started shortly after the disease has begun. At that time, physical and occupational therapy can help you keep your ability to straighten and bend your joints and maintain your daily life. […] Started early, treatments like phototherapy (light therapy) and medicines that work on the immune system like methotrexate and cyclosporine can help diminish scleroderma. […] Physical therapy can help you retain the ability to straighten and bend your joints. […] While there are many treatment options, the U.S. Food and Drug Administration (FDA) has not approved any drug to treat scleroderma on the skin. Still, there are effective treatments to treat the skin problems.

• #62 Scleroderma: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/scleroderma-treatment

  When the morphea goes deeper, UVA-1 phototherapy may be prescribed. UVA-1 produces long wavelengths that can deeply penetrate the skin to reduce hardness. It can also treat the itch. Some patients see complete clearing of their skin. […] Severe scleroderma that affects the skin and internal organs: For this, a dermatologist may recommend extracorporeal photopheresis (ECP). A study found that this treatment can be effective for severe scleroderma when ECP is started early. […] Treating the skin can prevent these changes. It can also reduce how much scleroderma you get on your skin, which can prevent skin hardening over joints. […] Treating your skin can also improve your quality of life. Most people feel better when they have less hardening and fewer signs or scleroderma on their skin.

• #63 Scleroderma: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/scleroderma-treatment

  When the morphea goes deeper, UVA-1 phototherapy may be prescribed. UVA-1 produces long wavelengths that can deeply penetrate the skin to reduce hardness. It can also treat the itch. Some patients see complete clearing of their skin. […] Severe scleroderma that affects the skin and internal organs: For this, a dermatologist may recommend extracorporeal photopheresis (ECP). A study found that this treatment can be effective for severe scleroderma when ECP is started early. […] Treating the skin can prevent these changes. It can also reduce how much scleroderma you get on your skin, which can prevent skin hardening over joints. […] Treating your skin can also improve your quality of life. Most people feel better when they have less hardening and fewer signs or scleroderma on their skin.

• #64 Scleroderma Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/report/scleroderma

  There is no cure for scleroderma. Many drugs that are useful for other autoimmune inflammatory disorders have not proven to be very effective for scleroderma. Experimental work is ongoing to develop procedures or to find drugs that can treat the underlying processes that cause damage. […] Researchers are investigating a possible benefit of transplanting the patient’s own stem cells (an autologous transplant). The transplant procedures introduce normal white blood cells that replace the abnormal autoimmune cells. […] Nitroglycerin is a quick acting nitrate and is used as an ointment to treat hardened skin. […] Phototherapy (light therapy) is now considered by some experts to be the treatment of choice for local scleroderma. […] Cyclophosphamide (Cytoxan), an immunosuppressive drug, may be effective for preventing lung deterioration and is an important medication for treating pulmonary fibrosis, particularly when given early in the course of the disease.

• #65 Scleroderma: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/scleroderma-treatment

  When the morphea goes deeper, UVA-1 phototherapy may be prescribed. UVA-1 produces long wavelengths that can deeply penetrate the skin to reduce hardness. It can also treat the itch. Some patients see complete clearing of their skin. […] Severe scleroderma that affects the skin and internal organs: For this, a dermatologist may recommend extracorporeal photopheresis (ECP). A study found that this treatment can be effective for severe scleroderma when ECP is started early. […] Treating the skin can prevent these changes. It can also reduce how much scleroderma you get on your skin, which can prevent skin hardening over joints. […] Treating your skin can also improve your quality of life. Most people feel better when they have less hardening and fewer signs or scleroderma on their skin.

• #66 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Opioids are also called narcotics. They include prescription medications used to relieve pain only. […] Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. […] Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. […] Cyclophosphamide is another immunosuppressant medication thats been shown to improve skin fibrosis in scleroderma. […] Methotrexate is another immunosuppressant thats been used for decades to treat inflammatory diseases, including rheumatoid arthritis. […] Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. […] People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux.

• #67 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Opioids are also called narcotics. They include prescription medications used to relieve pain only. […] Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. […] Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. […] Cyclophosphamide is another immunosuppressant medication thats been shown to improve skin fibrosis in scleroderma. […] Methotrexate is another immunosuppressant thats been used for decades to treat inflammatory diseases, including rheumatoid arthritis. […] Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. […] People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux.

• #68 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. […] Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. […] Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. […] Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. […] Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. […] People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. […] Pulmonary hypertension medications include: Ambrisentan, bosentan and macitentan, Sildenafil and tadalafil, Epoprostenol, iloprost, tresprostinil, Selexipag and riociguat. […] Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital.

• #69 Scleroderma Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/scleroderma.html

  Nonsteroidal anti-inflammatory drugs (NSAIDs) may be useful for joint and tendon inflammation. […] Diuretics encourage the body to release excess fluid as urine. […] Omeprazole (Prilosec) or related medications may be quite effective for heartburn related to esophageal disease. […] Other immune-suppressing medications that are sometimes recommended for scleroderma include azathioprine, tocilizumab, or rituximab. […] A promising experimental therapy for severe disease is high-dose immunosuppression with stem cell treatment, although serious side effects (including overwhelming infection and heart problems) have limited its use. […] Many patients find relief from Raynaud’s phenomenon with nonmedication approaches such as limiting their exposure to cold and by wearing warm clothing, especially mittens and socks.

• #70 Esophageal Scleroderma Treatments | Northwestern Medicine

  https://www.nm.org/conditions-and-care-areas/gastroenterology/esophageal-scleroderma/treatments

  There is no cure for esophageal or other types of scleroderma, but your physician can provide relief with certain treatments, including: […] Medications: Over-the-counter and prescription medication can control stomach acid production. Other medications promote better muscle activity in the esophagus. […] Pneumatic (balloon) dilation: Using an endoscope, a special balloon is passed through the mouth and into the esophagus, where it is inflated to stretch the restrictive muscles. […] Dietary changes: Eat slowly and chew thoroughly […] Eat soft or blended foods […] Avoid foods that stick in the throat […] Avoid alcohol and foods that cause gastroesophageal reflux disease (GERD), including greasy and fatty foods, spicy foods, chocolate, tobacco and caffeine […] Lifestyle changes: Stay upright after meals […] Raise the head of your bed […] Lose weight to reduce pressure on your abdomen […] Avoid tight-fitting clothing.

• #71 Esophageal Scleroderma Treatments | Northwestern Medicine

  https://www.nm.org/conditions-and-care-areas/gastroenterology/esophageal-scleroderma/treatments

  There is no cure for esophageal or other types of scleroderma, but your physician can provide relief with certain treatments, including: […] Medications: Over-the-counter and prescription medication can control stomach acid production. Other medications promote better muscle activity in the esophagus. […] Pneumatic (balloon) dilation: Using an endoscope, a special balloon is passed through the mouth and into the esophagus, where it is inflated to stretch the restrictive muscles. […] Dietary changes: Eat slowly and chew thoroughly […] Eat soft or blended foods […] Avoid foods that stick in the throat […] Avoid alcohol and foods that cause gastroesophageal reflux disease (GERD), including greasy and fatty foods, spicy foods, chocolate, tobacco and caffeine […] Lifestyle changes: Stay upright after meals […] Raise the head of your bed […] Lose weight to reduce pressure on your abdomen […] Avoid tight-fitting clothing.

• #72 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. […] Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. […] Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. […] Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. […] Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. […] People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. […] Pulmonary hypertension medications include: Ambrisentan, bosentan and macitentan, Sildenafil and tadalafil, Epoprostenol, iloprost, tresprostinil, Selexipag and riociguat. […] Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital.

• #73 Scleroderma | Conditions & Treatments | UR Medicine

  https://www.urmc.rochester.edu/conditions-and-treatments/scleroderma

  Gastrointestinal tract: Acid reducing medications are used to treat acid reflux (heart burn). If the movement of the intestines is affected, medications that control diarrhea and constipation are prescribed. […] Kidneys: A particular blood pressure medicine called an angiotensin converting enzyme inhibitor (ACE inhibitor) is effective in treating scleroderma involvement of the kidney. […] At UR Medicine, we have physician scientists who are actively studying the processes in the body that lead to the scarring found in scleroderma. Research such as this is important for developing improved therapies for scleroderma. […] Our researchers are studying new medications to improve treatment and quality of life of people with scleroderma.

• #74 Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2008/1015/p961.html

  In addition to therapies to control gastrointestinal symptoms and prevent GERD complications, patients with systemic sclerosis and gastric antral vascular ectasia may require endoscopic laser coagulation to decrease the risk of bleeding. […] Results from two recent randomized trials suggest that oral or intravenous cyclophosphamide is beneficial in patients with early and progressive interstitial lung disease. […] All patients with systemic sclerosis should be advised to check their blood pressure at home on a regular basis. Any persistent elevations should prompt a medical evaluation and treatment with ACE inhibitors if scleroderma renal crisis is suspected. ACE inhibitors should be used to control hypertension despite rising serum creatinine levels or the initiation of dialysis because they are essential for preserving and restoring renal function.

• #75 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  Mycophenolate mofetil (with a target dose of 1.5-3 g daily in 2 divided doses) has shown benefits in managing systemic sclerosis-related interstitial lung disease. […] When no contraindication exists, supplemental oxygen, diuretics, and anticoagulation are recommended, alongside patient education, a healthy lifestyle, and exercise as tolerated. […] ACE inhibitors represent the sole effective treatment for scleroderma renal crisis. Initiation should occur upon the earliest signs, with a maximum tolerated dose utilized.

• #76 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #77 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Proton pump inhibitors are available both over the counter and by prescription. These reduce stomach acid to control GI symptoms like heartburn and reflux. […] Scleroderma can affect your lower GI tract, causing symptoms like constipation or diarrhea. […] Medications to treat lung fibrosis include mycophenolate and cyclophosphamide, as well as some newer biologic agents. […] Nintedanib was just approved in 2019 to treat scleroderma-associated lung disease (SSc-ILD) and to reduce lung fibrosis. […] Tocilizumab was just approved in 2021 to treat scleroderma-associated lung fibrosis. […] People with scleroderma often develop serious forms of high blood pressure or hypertension that affect the lungs or kidneys. […] Pulmonary hypertension medications include: Ambrisentan, bosentan and macitentan, Sildenafil and tadalafil, Epoprostenol, iloprost, tresprostinil, Selexipag and riociguat. […] Captopril, an angiotensin-converting enzyme (ACE) inhibitor, is used to treat scleroderma renal crisis and lower your blood pressure in the hospital.

• #78 Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2008/1015/p961.html

  In addition to therapies to control gastrointestinal symptoms and prevent GERD complications, patients with systemic sclerosis and gastric antral vascular ectasia may require endoscopic laser coagulation to decrease the risk of bleeding. […] Results from two recent randomized trials suggest that oral or intravenous cyclophosphamide is beneficial in patients with early and progressive interstitial lung disease. […] All patients with systemic sclerosis should be advised to check their blood pressure at home on a regular basis. Any persistent elevations should prompt a medical evaluation and treatment with ACE inhibitors if scleroderma renal crisis is suspected. ACE inhibitors should be used to control hypertension despite rising serum creatinine levels or the initiation of dialysis because they are essential for preserving and restoring renal function.

• #79 Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2008/1015/p961.html

  In addition to therapies to control gastrointestinal symptoms and prevent GERD complications, patients with systemic sclerosis and gastric antral vascular ectasia may require endoscopic laser coagulation to decrease the risk of bleeding. […] Results from two recent randomized trials suggest that oral or intravenous cyclophosphamide is beneficial in patients with early and progressive interstitial lung disease. […] All patients with systemic sclerosis should be advised to check their blood pressure at home on a regular basis. Any persistent elevations should prompt a medical evaluation and treatment with ACE inhibitors if scleroderma renal crisis is suspected. ACE inhibitors should be used to control hypertension despite rising serum creatinine levels or the initiation of dialysis because they are essential for preserving and restoring renal function.

• #80 Systemic sclerosis

  https://dermnetnz.org/topics/systemic-sclerosis

  Pulmonary artery hypertension treatment includes vasodilatory therapies. […] Renal disease management includes monitoring blood pressure regularly, avoiding corticosteroids, and using angiotensin-converting enzyme inhibitors. […] Gastrointestinal tract treatment includes protein pump inhibitors, alginates, prokinetics, and nutritional supplements.

• #81 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Blood pressure medicine is prescribed to manage Raynauds phenomenon. These medications open constricted blood vessels. […] Phosphodiesterase-5 (PED-5) inhibitors are medications that can be used to treat more severe Raynauds phenomenon symptoms, such as ulcerations on the fingers. […] Topical nitroglycerine cream or paste can be used to treat mild, localized Raynauds symptoms like a skin ulcer. […] Pain is a common symptom for people living with scleroderma. You may have pain, stiffness or aches in your joints or muscles. Medications can help you manage pain, but they may have other side effects that could be serious. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes called anti-inflammatories. These are common pain medications. […] Steroids, also called glucocorticoids or corticosteroids, are stronger medications to treat flare-ups of inflammation that cause pain or joint swelling.

• #82 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Blood pressure medicine is prescribed to manage Raynauds phenomenon. These medications open constricted blood vessels. […] Phosphodiesterase-5 (PED-5) inhibitors are medications that can be used to treat more severe Raynauds phenomenon symptoms, such as ulcerations on the fingers. […] Topical nitroglycerine cream or paste can be used to treat mild, localized Raynauds symptoms like a skin ulcer. […] Pain is a common symptom for people living with scleroderma. You may have pain, stiffness or aches in your joints or muscles. Medications can help you manage pain, but they may have other side effects that could be serious. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes called anti-inflammatories. These are common pain medications. […] Steroids, also called glucocorticoids or corticosteroids, are stronger medications to treat flare-ups of inflammation that cause pain or joint swelling.

• #83 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Blood pressure medicine is prescribed to manage Raynauds phenomenon. These medications open constricted blood vessels. […] Phosphodiesterase-5 (PED-5) inhibitors are medications that can be used to treat more severe Raynauds phenomenon symptoms, such as ulcerations on the fingers. […] Topical nitroglycerine cream or paste can be used to treat mild, localized Raynauds symptoms like a skin ulcer. […] Pain is a common symptom for people living with scleroderma. You may have pain, stiffness or aches in your joints or muscles. Medications can help you manage pain, but they may have other side effects that could be serious. […] Nonsteroidal anti-inflammatory drugs (NSAIDs) are sometimes called anti-inflammatories. These are common pain medications. […] Steroids, also called glucocorticoids or corticosteroids, are stronger medications to treat flare-ups of inflammation that cause pain or joint swelling.

• #84 Treatments & Medications – National Scleroderma Foundation

  https://scleroderma.org/treatments-medications/

  Opioids are also called narcotics. They include prescription medications used to relieve pain only. […] Systemic medications target your immune system to treat skin fibrosis, the scarring of your skin. […] Mycophenolate is a commonly prescribed medication to help improve skin fibrosis in scleroderma. […] Cyclophosphamide is another immunosuppressant medication thats been shown to improve skin fibrosis in scleroderma. […] Methotrexate is another immunosuppressant thats been used for decades to treat inflammatory diseases, including rheumatoid arthritis. […] Intravenous immunoglobulin (IVIG) is an immunosuppressant medication that may improve scleroderma skin, muscle or joint symptoms. […] People with scleroderma often have gastrointestinal (GI) symptoms like heartburn or acid reflux.

• #85 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #86 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  Systemic sclerosis (SSc) is a connective tissue disease characterized by progressive fibrosis of the skin and internal organs and has significant clinical sequelae. Management of SSc cutaneous disease remains challenging and often is driven by extracutaneous manifestations. Methotrexate is the typical first-line therapy for patients with early progressive cutaneous disease. […] However, in patients with diffuse progressive skin disease and inflammatory arthritis, methotrexate or rituximab monotherapy should be considered. First-line therapy for patients with concomitant myositis includes methotrexate or intravenous immunoglobulin (IVIG). For patients with both cutaneous findings and interstitial lung disease, studies have suggested the efficacy of mycophenolate mofetil or rituximab. Second-line therapies, including UVA-1 phototherapy, IVIG, or rituximab, can be considered in patients with disease refractory to first-line treatments. […] Clinical trials investigating the utility of emerging therapies such as abatacept and tocilizumab in the treatment of SSc are under way, and preliminary results are promising. […] Overall, SSc is a clinically heterogenous disease that affects multiple organ systems. Providers should assess extracutaneous involvement and use evidence-based recommendations to select the most appropriate therapy for patients with SSc.

• #87 Scleroderma – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/scleroderma/diagnosis-treatment/drc-20351957

  There is no treatment that can cure or stop the overproduction of collagen that happens in scleroderma. But a variety of treatments can help control symptoms and prevent complications. […] Because scleroderma can affect so many different parts of the body, the choice of medicine varies depending on the symptoms. […] Medicines that suppress the immune system, such as those taken after organ transplants, may help reduce progression of some scleroderma symptoms, such as the thickening of the skin or worsening of lung damage. […] Physical or occupational therapists can help you improve your strength and mobility and maintain independence with daily tasks. […] Stem cell transplants might be an option for people who have serious symptoms that haven’t responded to more-common treatments. If the lungs or kidneys have been badly damaged, organ transplants might be considered.

• #88 Scleroderma: Diagnosis and treatment

  https://www.aad.org/public/diseases/a-z/scleroderma-treatment

  There is currently no cure for scleroderma. However, treatment can improve a person’s quality of life. […] Treatment works best when started early. If the diagnosis is scleroderma, treatment is most effective when started shortly after the disease has begun. At that time, physical and occupational therapy can help you keep your ability to straighten and bend your joints and maintain your daily life. […] Started early, treatments like phototherapy (light therapy) and medicines that work on the immune system like methotrexate and cyclosporine can help diminish scleroderma. […] Physical therapy can help you retain the ability to straighten and bend your joints. […] While there are many treatment options, the U.S. Food and Drug Administration (FDA) has not approved any drug to treat scleroderma on the skin. Still, there are effective treatments to treat the skin problems.

• #89 Rehabilitation, Therapy Goals for Scleroderma, Acroosteolysis – The Rheumatologist

  https://www.the-rheumatologist.org/article/rehabilitation-therapy-goals-for-scleroderma-acroosteolysis/

  Scleroderma is a rare rheumatologic autoimmune disease that affects the skin and can also affect other organs. […] Theres no cure for scleroderma; however, many patients benefit from physical and occupational therapy to address impairments commonly associated with the disease. […] The main goal of therapy for patients with scleroderma is to retain range of motion. Because the skin can become taut, the patient may experience a permanent loss in range of motion.

• #90 Emerging treatments for scleroderma/systemic sclerosis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8170563/

  New trials and increased understanding of pathogenesis have produced a number of promising therapies for SSc-related skin disease. […] Methotrexate is the most commonly administered immunosuppressive in patients with SSc and has been shown to be effective in multiple prospective trials. […] The use of MMF in treating SSc is supported by several small observational studies. […] Intravenous immunoglobulin (IVIG) is used to treat a host of autoimmune diseases and its use in patients with SSc was first reported in 2000 in three patients with rapidly progressing skin disease. […] Rituximab is a chimeric monoclonal antibody that targets CD20, a B-lymphocyte surface molecule that assists in differentiation and development of B cells into plasma cells. […] UVA-1 (340-400 nm) phototherapy has been used in various T cell-mediated diseases, such as atopic dermatitis and mycosis fungoides, as it is thought to penetrate deep into the dermis to induce apoptosis of infiltrating T cells. […] The use of tocilizumab, an interleukin 6 receptor alpha inhibitor, has previously been studied, and phase III studies are under way. […] As such, abatacept, a medication that interferes with the activation of T cells, is thought to be a promising therapeutic for the treatment of SSc.

• #91 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  In patients with systemic sclerosis-related interstitial lung disease, a clinically meaningful decline of 10% or more in lung function was seen in 24.5% of placebo recipients, compared with 8.6% of tocilizumab recipients. […] Other agents are currently being studied for skin and lung involvement. For example, trials of rituximab have yielded promising results, with improvement of skin fibrosis and prevention of worsening lung fibrosis. […] The tyrosine kinase inhibitor nintedanib is approved for slowing the rate of decline in pulmonary function in adults with systemic sclerosis-related interstitial lung disease. […] Phototherapy using longer-wavelength ultraviolet A (UVA) light (ie, UVA1, 340-400 nm) has proved beneficial for cutaneous lesions in scleroderma. […] Hematopoietic stem cell transplantation (HSCT) has been shown to be effective. However, it is associated with a high rate of procedure-related complications.

• #92 EULAR recommendations for the treatment of systemic sclerosis: 2023 update | Annals of the Rheumatic Diseases

  https://ard.bmj.com/content/early/2024/10/17/ard-2024-226430

  PDE5 inhibitors should also be considered for treatment of SSc-RP. […] Intravenous iloprost should be considered for severe SSc-RP following failure of oral therapy. […] PDE5 inhibitors and/or intravenous iloprost should be considered for the treatment of DU in patients with SSc. […] Combination of PDE-5i and ERAs should be considered as first-line treatment of SSc-PAH. […] Intravenous epoprostenol should be considered for the treatment of SSc patients with advanced PAH (classes III and IV). […] Nintedanib should be considered alone or in combination with MMF for the treatment of SSc ILD. […] Tocilizumab should be considered for the treatment of SSc-ILD. […] High-intensity immunosuppression (usually including cyclophosphamide) followed by autologous haematopoietic stem cell transplantation (HSCT) may be considered for the treatment of selected patients with early severe dcSSc and poor prognosis, in the absence of advanced cardiorespiratory involvement. […] MTX should be considered for the treatment of musculoskeletal involvement in SSc.

• #93 State-of-the-art evidence in the treatment of systemic sclerosis | Nature Reviews Rheumatology

  https://www.nature.com/articles/s41584-023-00909-5

  Treatment of systemic sclerosis (SSc) is organ-based or aimed at disease modification. Autologous haematopoietic stem cell transplantation can improve survival in patients with early diffuse cutaneous SSc who are at high risk of mortality, such as those with very high skin scores (as measured by the modified Rodnan skin score) or moderate skin involvement and worsening interstitial lung disease (ILD). Immunosuppressives and some biologic agents can soften skin and change the natural history of early diffuse cutaneous SSc. Appropriate treatment for patients with early limited cutaneous SSc is unknown, and further research is needed. ILD is usually treated by the use of mycophenolate mofetil as the initial therapy and then other immunosuppressives or biologic agents, but if ILD is fibrotic and progressing, anti-fibrotic therapy can be added, such as nintedanib (and possibly pirfenidone). Raynaud phenomenon in SSc is treated with calcium channel blockers and then phosphodiesterase 5 inhibitors or intravenous iloprost.

• #94 Systemic Sclerosis (Scleroderma) Treatment With CAR T-Cell Therapy | Kyverna

  https://kyvernatx.com/patients/systemic-sclerosis/

  SSc has limited treatment options which include immunosuppressive agents for early-stage disease, while those who progress or who are at higher risk of death may receive autologous hematopoietic stem cell transplantation which can improve survival but is associated with risk of morbidity and mortality. […] CAR T-cell therapy involves modifying a patients T cells to recognize and remove B cells in the patients body. […] We believe our approach may present a significant advantage over current standard-of-care therapies by aiming to directly deplete B cells and potentially resetting disease-contributing B cells. […] Participation in clinical trials is the best way to access our investigational product. Kyverna may consider requests for expanded access when alternative therapy options have been exhausted.

• #95 Systemic Sclerosis (Scleroderma) Treatment With CAR T-Cell Therapy | Kyverna

  https://kyvernatx.com/patients/systemic-sclerosis/

  SSc has limited treatment options which include immunosuppressive agents for early-stage disease, while those who progress or who are at higher risk of death may receive autologous hematopoietic stem cell transplantation which can improve survival but is associated with risk of morbidity and mortality. […] CAR T-cell therapy involves modifying a patients T cells to recognize and remove B cells in the patients body. […] We believe our approach may present a significant advantage over current standard-of-care therapies by aiming to directly deplete B cells and potentially resetting disease-contributing B cells. […] Participation in clinical trials is the best way to access our investigational product. Kyverna may consider requests for expanded access when alternative therapy options have been exhausted.

• #96 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  Current treatment of systemic sclerosis is directed toward managing complications and providing symptomatic relief. In addition, a range of disease-modifying treatments have been investigated. […] Disease-modifying treatment aims at inhibiting tissue fibrosis and vascular and immune system alterations, which are the three crucial components of disease pathogenesis. […] Therapies targeting cytokine signalling, including interleukin-6 (IL-6), and other immune-inflammatory therapies, have produced promising results. […] To date, the US Food and Drug Administration (FDA) has approved nintedanib and as well as tocilizumab for refractory, progressive interstitial lung disease due to systemic sclerosis. […] A phase 3 trial of tocilizumab in systemic sclerosis failed to meet its primary endpoint of improvement in modified Rodnan skin score; however, tocilizumab therapy did result in preservation of lung function.

• #97 Systemic sclerosis

  https://dermnetnz.org/topics/systemic-sclerosis

  Treatments help with symptoms and may modify the disease outcome, especially early in the disease course. They focus on suppressing inflammation and dilating abnormal / constricted blood vessels. Some newer treatments target specific immunological pathways and signalling molecules. […] Hydroxychloroquine may help these constitutional symptoms. […] Mycophenolate mofetil, Methotrexate, Cyclophosphamide, Corticosteroids (with caution), Rituximab (B-cell depletion therapy) may be useful for lung and skin. […] Nintedanib (tyrosine kinase inhibitors) now approved for SSc associated Interstitial Lung Disease in the US and Europe; no proven impact on skin sclerosis. […] Tocilizumab (anti-interleukin (IL) 6 therapy) shows a trend towards improvement for lung and skin in early progressive and inflammatory diffuse cutaneous SSc.

• #98 Systemic sclerosis

  https://dermnetnz.org/topics/systemic-sclerosis

  Abatacept (anti-cytotoxic T-cell lymphocyte-associated inhibitor [CTLA] 4 therapy shows a possible trend towards skin improvement in inflammatory disease. […] Autologous stem cell transplant for severe and acute progressive disease in selected patients seen within specialist centres. […] Intravenous immunoglobulin may be useful for skin and gastrointestinal tract. […] Tofacitinib well-tolerated, prevents bleomycin-induced skin fibrosis in a mouse model; a small study showed no clinical improvement in skin sclerosis, but larger studies are needed. […] Endothelin 1 antagonists (bosentan), Phosphodiesterase-5 inhibitors (tadalafil), Guanylate cyclase agonists (riociguat), Prostacyclin agonists (epoprostenol; selexipag) are used for vasodilation. […] General measures such as keeping warm, stretching exercises for joints to reduce the risk of worsening contractures and microstomia, and specifically-related physiotherapy can all be beneficial.

• #99 Systemic sclerosis

  https://dermnetnz.org/topics/systemic-sclerosis

  Abatacept (anti-cytotoxic T-cell lymphocyte-associated inhibitor [CTLA] 4 therapy shows a possible trend towards skin improvement in inflammatory disease. […] Autologous stem cell transplant for severe and acute progressive disease in selected patients seen within specialist centres. […] Intravenous immunoglobulin may be useful for skin and gastrointestinal tract. […] Tofacitinib well-tolerated, prevents bleomycin-induced skin fibrosis in a mouse model; a small study showed no clinical improvement in skin sclerosis, but larger studies are needed. […] Endothelin 1 antagonists (bosentan), Phosphodiesterase-5 inhibitors (tadalafil), Guanylate cyclase agonists (riociguat), Prostacyclin agonists (epoprostenol; selexipag) are used for vasodilation. […] General measures such as keeping warm, stretching exercises for joints to reduce the risk of worsening contractures and microstomia, and specifically-related physiotherapy can all be beneficial.

• #100 Get Scleroderma Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/scleroderma-treatment

  So, when you hear you may have scleroderma, youll want to find healthcare providers with lots of experience treating it. […] Your provider may recommend a combination of therapies to manage scleroderma symptoms. Medications, physical therapy and lifestyle changes may reduce pain and help you move more comfortably. […] Once we get your test results back, your provider will go over them with you. And then theyll work with you to build a highly personalized treatment plan. This plan will focus on the type of scleroderma you have, your symptoms and your recovery goals. Therapies could include: […] Several medications can help manage severe symptoms, especially if scleroderma affects many of your organs. […] Your provider may suggest you participate in one of our clinical trials if other therapies arent working. Enrolling in a clinical trial lets you try new medications that arent widely available. […] A scleroderma diagnosis is serious, but treatable. Cleveland Clinics expert healthcare providers can help you find the best ways using the latest treatments to manage your symptoms, so you can live an active, healthy and productive life.

• #101 What is Scleroderma? Types, Symptoms and Treatment Explained

  https://mydoctor.kaiserpermanente.org/mas/news/what-is-scleroderma-rheumatologist-explains-types-symptoms-and-treatment-2019538

  Patients are also encouraged to avoid certain medications such as steroids, and to monitor blood pressure so it stays in healthy ranges. Maintaining a healthy diet, managing stress and exercising can also help alleviate symptoms. Many patients benefit from physical therapy to ease joint stiffness and pain. […] Because scleroderma can affect so many different parts of the body, many medical specialists are often involved in a patient’s care, including a rheumatologist, nephrologist, dermatologist, cardiologist, pulmonologist and gastroenterologist. […] Although scleroderma is a chronic and often complex condition, working closely with a team of doctors can help patients manage symptoms and help prevent complications.

• #102 Scleroderma Treatment & Management: Approach Considerations, Pruritus, Raynaud Phenomenon

  https://emedicine.medscape.com/article/331864-treatment

  Recommendations regarding activity include the following: Ensure that the patient maintains a core body temperature to try to minimize the occurrence of Raynaud phenomenon episodes. […] All patients with systemic sclerosis should be treated in conjunction with an experienced rheumatologist who has a full understanding of the disease, the complications of the therapies, and the frequently serious adverse effects. […] Evaluate the patient every 3-6 months, depending on the disease activity and progression.

• #103 Scleroderma and Systemic Sclerosis (SSc): An Overview – HSS.edu

  https://www.hss.edu/conditions_scleroderma-and-systemic-sclerosis-overview.asp

  Scleroderma is a group of conditions affecting approximately 300,000 people in the United States. […] SSc is a chronic condition that should be treated and monitored by a rheumatologist, and frequently needs the input from physicians in other medical specialties. […] Treatment for this condition includes steroid and vitamin D creams, light (UVA1 or UVB) therapy, and immunosuppressive medications (corticosteroids, methotrexate, and mycophenalate mofetil). […] Although there is no cure for SSc, there are many treatments which can improve the various symptoms of the disease. […] The care of individuals with scleroderma frequently requires a team approach. Depending on your symptoms, you will likely also need to be referred to other specialists including pulmonologists, cardiologists, gastroenterologists, nephrologists, or dermatologists. The treatment depends on the organ system involvement.

• #104 Get Scleroderma Treatment | Cleveland Clinic

  https://my.clevelandclinic.org/services/scleroderma-treatment

  So, when you hear you may have scleroderma, youll want to find healthcare providers with lots of experience treating it. […] Your provider may recommend a combination of therapies to manage scleroderma symptoms. Medications, physical therapy and lifestyle changes may reduce pain and help you move more comfortably. […] Once we get your test results back, your provider will go over them with you. And then theyll work with you to build a highly personalized treatment plan. This plan will focus on the type of scleroderma you have, your symptoms and your recovery goals. Therapies could include: […] Several medications can help manage severe symptoms, especially if scleroderma affects many of your organs. […] Your provider may suggest you participate in one of our clinical trials if other therapies arent working. Enrolling in a clinical trial lets you try new medications that arent widely available. […] A scleroderma diagnosis is serious, but treatable. Cleveland Clinics expert healthcare providers can help you find the best ways using the latest treatments to manage your symptoms, so you can live an active, healthy and productive life.

• #105 Scleroderma Treatment Center – NYC | ColumbiaDoctors

  https://www.columbiadoctors.org/specialties/rheumatology/scleroderma-center

  The Scleroderma Center is dedicated to intensive research, the advancement of scleroderma treatments, and the understanding of its causes. […] At the Scleroderma Center, our rheumatologists are deeply committed to scleroderma research and to the discovery of new therapies to help our patients. […] Our physician-scientists are dedicated to finding better treatments for patients with scleroderma, understanding and identifying the cause of scleroderma, and investigating new techniques to effectively manage this illness. […] Our patients have access to a number of clinical trials that are available only at the Scleroderma Center. We are continually striving to better understand scleroderma, and translate our discoveries directly into better patient care.

• #106 Systemic Sclerosis (Scleroderma) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK430875/

  No definitive treatment or universally accepted disease-modifying agent can alter the natural course of the disease. However, managing the affected system or systems has proven effective. Early diagnosis is crucial for achieving improved outcomes. Clinical evaluation and identification of affected organs and disease progression are critical for treatment efficacy. Furthermore, treatment goals must be holistic and tailored to optimize the quality of life for affected patients while also preventing further organ damage. Patient education about the disease and encouragement to engage in regular exercise, maintain a healthy diet and lifestyle, and seek emotional support should be considered for every individual with systemic sclerosis. […] Several agents have been investigated for different manifestations of systemic sclerosis, yet a generalized lack of large randomized controlled trials undermines their efficacy. Cyclophosphamide (for lung disease and skin disease), mycophenolate mofetil (for lung disease and skin disease), methotrexate (MTX; for skin disease, inflammatory arthritis, and myositis), azathioprine (for skin disease, lung disease, and myositis), and hydroxychloroquine (HCQ; for skin disease) stand out as some of the most frequently utilized immunosuppressive agents.

• #107 Functional Medicine Scleroderma: Effective Treatment Strategies | Maggie Yu MD, IFMCP

  https://drmaggieyu.com/blog/functional-medicine-scleroderma-effective-treatment-strategies/

  Early detection and treatment of scleroderma can significantly improve outcomes and quality of life. […] Functional Medicine Offers Holistic Care: This approach looks at the root causes of scleroderma, offering personalized and comprehensive treatment plans. […] Natural Treatments Can Help: Integrating natural remedies, such as dietary changes and supplements, can alleviate symptoms and support overall health. […] Lifestyle Changes Matter: Simple modifications like stress management, regular exercise, and a balanced diet can provide relief and improve daily living. […] Collaboration with Specialists: Working with healthcare providers who understand functional medicine can lead to better management of the disease. […] Patient Education Empowers: Understanding the disease, its symptoms, and treatment options allows patients to take an active role in their health care.

• #108 Scleroderma – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/scleroderma/symptoms-causes/syc-20351952

  While there is no cure for scleroderma, treatments can ease symptoms, slow progression and improve quality of life. […] There are medicines that may help slow the progression of this lung damage. […] Prompt treatment of this condition is important to preserve kidney function.

• #109 Advances in Scleroderma Treatment: FAQ | Cedars-Sinai

  https://www.cedars-sinai.org/blog/treating-scleroderma.html

  A clinical trial with a similar drug called iloprost has been recently completed. If the results of the phase III clinical trial demonstrate the medication is safe and effective, we are hoping this drug will gain FDA approval and may become available for all scleroderma patients across the U.S. […] Scleroderma is not a disease affecting uniformly one organ—it cannot be tackled with a „one problem, one answer” approach. To effectively manage scleroderma, it is critical to assess, at the time of diagnosis, which internal organs are affected and the extent of their damage. […] Every patient is unique and requires tailored treatment. This approach requires a dedicated comprehensive program and a network of specialty physicians. Cedars-Sinai is one of the few centers in the country with highly specialized pulmonologists, cardiologists, gastroenterologists, plastic surgeons, dermatologists, nurses, patient coordinators and research teams working together to deliver the best possible care to our patients.

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