Materiały źródłowe

• #1 Interstitial lung disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/diagnosis-treatment/drc-20353113

  Lung scarring that already has occurred in interstitial lung disease can’t be reversed, and treatment won’t always stop the disease from getting worse. Some treatments may make symptoms better for a short time or slow the disease. Others help maintain quality of life. […] Because many of the different types of scarring diseases have no approved or proven therapies, clinical trials may be an option to get an experimental treatment. […] Intense research to find treatment options for specific types of interstitial lung disease is ongoing. Treatment may vary depending on the cause of ILD and what damage has happened in the lungs. Using on the latest scientific evidence, your healthcare professional may recommend: […] Corticosteroid medicines. At first, many people diagnosed with ILD are treated with a corticosteroid, namely prednisone (Prednisone Intensol, Rayos). Sometimes people are treated with other drugs that suppress the immune system. Depending on the cause of ILD, these medicines may slow or even keep the disease from getting worse.

• #1 Management – Lung Foundation Australia

  https://lungfoundation.com.au/health-professionals/conditions/interstitial-lung-disease/management/

  There is no cure for many types of ILD, but there are various treatment options and management strategies to help stop or slow the progression of the condition and manage symptoms. […] The following information is a general overview of some of the treatment and management options for those diagnosed with ILD. […] The use of medications for management of interstitial lung disease (ILD) is informed by the cause of the ILD. […] Establishing the aims of treatment is also important. In patients with ILD who require treatment with medication, the aims include improving or stabilising disease, or slowing disease progression. […] Broadly speaking, there are three categories of medications for ILD: (i) immunosuppressant; (ii) antifibrotic; and (iii) those for management of symptoms. […] Immunosuppressive therapy is generally used in ILD that is driven by the process of inflammation; for example, in autoimmune diseases or sarcoidosis.

• #1 Basics of Interstitial Lung Diseases | ILD Collaborative

  https://www.ildcollaborative.org/resources/basics-of-ilds

  How are ILDs treated? […] The treatment for an ILD depends on whether the cause is known or unknown. When an ILD is caused by breathing in chemicals, animal and vegetable dust, metals, smoke, asbestos, etc., the first and most important step is to avoid or minimize exposure to these substances. If the ILD is caused by exposure to a medication, doctors may switch the person to a different medication. […] Treatment for ILDs also depends on whether the ILD is primarily one that causes inflammation in the interstitium, or primarily one that causes fibrosis (scarring) in the interstitium. […] An ILD that primarily causes inflammation in the interstititum can be treated by calming the immune system down. Anti-inflammatory drugs, like steroids, or immune suppressant drugs can be used to treat ILDs in this category.

• #1

  https://www.pneumon.org/What-is-new-in-the-treatment-of-interstitial-lung-ndiseases,161867,0,2.html

  Interstitial lung diseases (ILDs) comprise an heterogenous group of more than 200 pulmonary disorders characterized by lung architectural distortion, with variable amount of fibrotic and/or inflammatory lesions, and associated with significant morbidity and mortality. […] Pharmacological management is driven by lumping ILDs according to the presumptive predominant pathogenic mechanism, although the coexistence of inflammation and fibrosis may be challenging. In inflammation-predominant diseases, corticosteroids and immunomodulatory agents are the mainstay of treatment. […] The use of antifibrotics may decelerate PPF worsening, similar to their beneficial effects on IPF, and treatment with nintedanib is suggested in patients with PFF, when standard management for fibrotic ILD has failed.

• #1 Interstitial Lung Disease (ILD) Treatment | Froedtert & MCW

  https://www.froedtert.com/interstitial-lung-disease

  Although it does not currently have a cure, ILD is treated based on the kind of lung disease you have and other co-morbidities that might exist. We tailor the therapy for your ILD based on the extent/severity of your lung disease, medical history, age, and your personal preferences for your health care. Examples of available therapies include: […] Corticosteroids: Oral corticosteroids (i.e. Prednisone) are the mainstay of treatment for many ILDs. They are used as mono-therapy or can be used in conjunction with other steroid-sparing agents. […] Steroid sparing agents: These are medications that tone-down your immune system, decreasing inflammation and scarring in the lungs. Common medications may include Azathioprine, Mycophenolate Mofetil and Methotrexate. […] Anti-fibrotic medications: A class of medications that slow (or, sometimes, halt) scarring (fibrosis). These include Pirfenidone and Nintedanib. […] Investigative medications: We participate in clinical trials evaluating the use of new medications in ILD. […] Oxygen therapy […] Pulmonary rehabilitation […] Smoking cessation […] Diagnosing and treating acid reflux (heart burn) […] Lung transplant.

• #1 Interstitial Lung Disease Medications | Treatment for ILD

  https://www.nationaljewish.org/conditions/medications/interstitial-lung-disease

  Many medications are used to treat interstitial lung disease (ILD) and its various symptoms. The goals of medication treatment are to make breathing more productive, reduce inflammation, and suppress overactive immune systems. […] Prednisone or some other form of corticosteroid, is often the first medication used for many forms of ILD. For some people, corticosteroids alone may decrease lung inflammation and cause an improvement in symptoms. […] Mycophenolate can be used to help reduce the amount of steroids required. It works to prevent the immune system from attacking cells in the body that result in fibrosis. […] Azathioprine is another medication that may be used to help reduce the amount of steroids required. […] Cyclophosphamide may be used if steroid therapy has failed to be effective or if corticosteroid treatment is not possible.

• #1

  https://www.pulmonaryfibrosis.org/understanding-pff/treatment-options/medications

  Tocilizumab is a biologic medication that is used to prevent and treat inflammation by interrupting the inflammatory process. It has been shown to reduce the rate of decline in pulmonary function in adult patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). […] Prednisone is used to treat and prevent inflammation by weakening the immune system. While prednisone is not usually used to treat idiopathic pulmonary fibrosis, it is sometimes used to treat inflammation in the lungs of people living with other forms of pulmonary fibrosis. […] Similar to prednisone, mycophenolate mofetil (MMF) also can treat and prevent inflammation by suppressing the immune system. MMF is sometimes used in combination with prednisone, but is also used alone. […] Azathioprine is used to suppress the immune system similarly to MMF. Several other anti-inflammatory therapies such as methotrexate, cyclophosphamide, cyclosporine, rapamycin (sirolimus), and tacrolimus have been used to treat different forms of interstitial lung disease. As mentioned previously, treatment with these agents is often tailored to specific diseases or forms of ILD and pulmonary fibrosis.

• #1 Interstitial Lung Disease: Stages, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17809-interstitial-lung-disease

  Theres no cure for interstitial lung disease. Treatment for ILD usually focuses on treating underlying disease and improving your symptoms. Your healthcare provider might prescribe physical therapy, supplemental oxygen or medication to reduce inflammation or slow down the disease. […] Corticosteroids. Drugs like prednisone can help reduce inflammation. […] Anti-fibrotic and cytotoxic drugs. These medications can slow down lung scarring. They include azathioprine, cyclophosphamide, pirfenidone and nintedanib. […] Biologic drugs. Medications like rituximab are sometimes used to treat autoimmune diseases and other causes of ILD. […] Treatment for GERD. Gastroesophageal reflux disease (GERD) can make ILD worse, so your provider may prescribe medications to keep stomach acid down. […] Pulmonary rehabilitation. Breathing exercises and physical therapy can make your lungs stronger and breathing easier.

• #1 Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD) Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1343513-treatment

  For patients with CTD-ILD other than SSc-ILD, the ACR/ACCP guidelines conditionally recommend azathioprine, mycophenolate, rituximab, and cyclophosphamide as initial corticosteroid-sparing treatments, with consideration of tocilizumab in selected conditions if progression occurs. […] Cyclophosphamide, an alkylating agent, can be used with or without steroids in the treatment of SSc. […] Mycophenolate mofetil is associated with several adverse effects, such as diarrhea (most common), bone marrow suppression, and progressive multifocal leukoencephalopathy. […] Guidelines from the American Thoracic Society strongly recommend mycophenolate for the treatment of patients with SSc-ILD. […] Tocilizumab, an anti-IL-6 inhibitor, has shown promise for the treatment of ILD due to scleroderma. […] Hematopoietic stem cell transplantation (HSCT) has emerged as a novel approach to the management of advanced SSc.

• #1 Basics of Interstitial Lung Diseases | ILD Collaborative

  https://www.ildcollaborative.org/resources/basics-of-ilds

  An ILD that primarily causes fibrosis (scarring) in the interstitium can be treated with anti-fibrotic medications. For example, idiopathic pulmonary fibrosis (IPF) causes fibrosis (scarring) and can be treated with nintedanib or pirfenidone. A person with an ILD that primarily causes fibrosis will not be helped by anti-inflammatory drugs or immune suppressant drugs.

• #1

  https://www.pneumon.org/What-is-new-in-the-treatment-of-interstitial-lung-ndiseases,161867,0,2.html

  Antifibrotics were initially launched in IPF. […] Based on the fact that pulmonary fibrosis is an end stage situation requiring therapy to hinder disease progression, there has been much interest in whether antifibrotics could be beneficial in PPF. […] Nintedanib, originally designed as an anti-cancer drug, is a tyrosine kinase inhibitor that binds to several growth factor receptors, resulting in attenuation of neo-angiogenesis and inhibition of several pro-fibrotic effects. […] Pirfenidone is a synthetic pyridine that inhibits fibrotic, inflammatory and oxidation pathways, through downregulation of inflammatory cytokines and pro-fibrotic factors, including TGF- and tumor necrosis factor-a (TNF-a). […] The latest recommendations for SSc-ILD suggest that cyclophosphamide is implemented in case of disease progression, while mycophenolate mofetil (MMF) use is neither recommended nor discouraged.

• #1 Interstitial lung disease – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/interstitial-lung-disease/diagnosis-treatment/drc-20353113

  Medicines that slow the worsening of idiopathic pulmonary fibrosis. Pirfenidone (Esbriet) and nintedanib (Ofev) are medicines that may slow the rate at which IPF worsens. Ofev also has been approved for people with lung fibrosis that’s getting worse due to other types of interstitial lung disease. Side effects for both drugs are common. Talk with your healthcare professional about the pros and cons of these medicines. […] Oxygen therapy. Using oxygen can’t stop lung damage, but it can: Make it easier to breathe and exercise. Prevent or lessen complications from low blood oxygen levels. Lower blood pressure in the right side of your heart. Make your sleep and sense of well-being better. […] The aim of pulmonary rehabilitation is to make you better able to function and live a full, satisfying life. That’s why pulmonary rehabilitation programs focus on: Learning more about your lung disease. Exercise, so you can become more physically active for longer periods of time. Breathing techniques that make your lungs more efficient. Emotional support. Nutritional counseling. […] A lung transplant may be an option of last resort for some people with severe interstitial lung disease when other treatment options haven’t helped.

• #1

  https://www.pulmonaryfibrosis.org/understanding-pff/treatment-options/medications

  Drug therapy for pulmonary fibrosis is often specific for the type of PF a patient has. It is important to discuss drug therapy with your physician to learn what medications may be appropriate to treat your form of pulmonary fibrosis. Some common therapies (but not all potential therapies) are listed below. […] Nintedanib is an anti-fibrotic drug that is approved in the United States to treat idiopathic pulmonary fibrosis, scleroderma-associated ILD (SSc-ILD), and chronic interstitial lung diseases in which fibrosis continues to progress. In clinical trials, nintedanib has been shown to slow the decline in lung function in SSc-ILD, progressive fibrosing ILD, and mild-to-moderate IPF. […] Pirfenidone is an antifibrotic and anti-inflammatory drug approved to treat IPF in the US, Europe, Canada, Asia, and Australia. In clinical trials, pirfenidone has been shown to slow progression of mild-to-moderate idiopathic pulmonary fibrosis.

• #1 Management – Lung Foundation Australia

  https://lungfoundation.com.au/health-professionals/conditions/interstitial-lung-disease/management/

  High doses of prednisolone (including intravenous methylprednisolone) may be used in conditions which are predominantly inflammatory, in which case, improvement is the aim of treatment. […] After the initial phase, the aim of immunosuppressive treatment is to halt progression and stabilise disease. […] Anti-fibrotic therapy is currently recommended in mild-to-moderate IPF and PPF; Current anti-fibrotic therapies (Nintedanib (OfevTM) and Pirfenidone (EsbrietTM)) are aimed at slowing disease progression. […] Nintedanib or Pirfenidone are recommended in the treatment of mild-to-moderate Idiopathic Pulmonary Fibrosis (IPF), whereas only Nintedanib is recommended in the treatment of PPF. […] In general, a patient on antifibrotic therapy would lose a mean of about 120ml of lung function a year, compared to someone not on treatment who would lose about 240ml of lung function a year.

• #1 Pharmacological Treatment of Interstitial Lung Diseases: A Novel Landscape for Inhaled Agents

  https://www.mdpi.com/1999-4923/16/11/1391

  Similarly, inhaled pirfenidone has been tested in early studies; in the ATLAS study, a phase 1b trial, the safety and efficacy of inhaled pirfenidone (AP01) at a dose of 50 mg once per day or 100 mg two times per day were assessed in IPF patients who were intolerant, reluctant, or ineligible for oral pirfenidone or nintedanib. […] Alongside a potential reduction in adverse events, inhalation of antifibrotic agents can result in more effective antifibrotic properties possibly slowing the pace of FVC decline. […] Treprostinil, a prostacyclin analog, may be considered in patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD) based on the findings from the INCREASE study. […] The phase 3 trial evaluating the efficacy of inhaled treprostinil in patients with IPF is ongoing; results are expected by 2025. […] Remarkable progress has been achieved in the treatment of ILDs. […] Investigation of novel molecules and formulations is currently ongoing in new recruiting trials.

• #1 Interstitial Lung Disease Symptoms & Causes | Baptist Health

  https://www.baptisthealth.com/care-services/conditions-treatments/interstitial-lung-disease

  Antibiotics may be used to treat interstitial lung disease that is caused by an infection. Antibiotics can resolve infections and reduce inflammation. You can also use antibiotics to treat fungal infections that may cause interstitial lung disease. You might take antibiotics temporarily or long term. […] Certain medications can slow down your immune system. Suppressing your immune system can help reduce inflammation and prevent the progression of interstitial lung disease. Immune suppression medication might not directly treat your condition. Instead, it may help to prevent the disease from getting worse. […] If your interstitial lung disease is severe, your doctor may recommend a complete lung transplant. A lung transplant is a major surgery. It is usually only recommended for people who have an interstitial lung disease that is not responding to other treatment options. After a lung transplant, many people report a higher quality of life.

• #1 Interstitial Lung Disease (ILD)

  https://www.webmd.com/lung/interstitial-lung-disease

  Corticosteroids. In some forms of ILD, inflammation in your lungs causes damage and scarring. Corticosteroids can slow down your immune system. This lessens the amount of inflammation in your lungs and the rest of your body. […] N-acetylcysteine (Mucomyst). This potent antioxidant may slow the decline of lung function in some forms of ILD. You’ll take it in combination with other treatments. […] Drugs that are considered controversial for ILD treatment include: Cyclophosphamide (Cytoxan), Cyclosporine, Methotrexate, Nintedanib (Ofev), Pirfenidone (Esbriet). These medicines affect the way your immune system works. If your doctor thinks you need one, they’ll keep a close watch on you while you’re taking it. These medications can have serious side effects.

• #1 Interstitial Lung Disease Medications | Treatment for ILD

  https://www.nationaljewish.org/conditions/medications/interstitial-lung-disease

  Pirfenidone is an oral medication taken three times daily. […] Nintedanib is an oral medication taken twice daily. […] The decision to treat people with ILD involves a careful weighing of the potential risks and benefits of therapy. The potential benefits from the treatment usually outweigh the risk from the medication side effects.

• #1 Interstitial Lung Disease: Stages, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17809-interstitial-lung-disease

  Oxygen therapy. Your provider will prescribe extra oxygen if you dont have enough getting to your blood or tissues. Its delivered through a mask or tube in your nose. […] Lung transplant. Some people with severe cases of ILD get a lung transplant. […] Some treatments for interstitial lung disease can weaken your immune system. Your provider will monitor you closely for complications if youre taking one of these medications.

• #1 Interstitial Lung Diseases – Treatment | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/interstitial-lung-diseases/treatment

  Your treatment for ILDs will depend on the cause of your condition and how serious your symptoms are. Your healthcare provider will work with you to decide on a treatment plan for you. […] Treatment for ILDs does not cure your lung damage, but it can prevent lung damage from getting worse and can help you breathe easier. […] Depending on the type of ILD you have, your doctor may prescribe medicine to help you breathe easier. […] Oxygen therapy is a treatment that delivers oxygen gas for you to breathe. […] Oxygen therapy can be given for a short or long period of time in the hospital, another medical setting, or at home. […] Pulmonary rehabilitation is a supervised program that includes exercise training, health education, and breathing techniques for people who have certain lung conditions, lung problems due to other conditions, or after a lung transplant. […] Lung transplant is surgery to remove a diseased lung and replace it with a healthy lung. […] Lung transplants are used to improve quality of life and extend the lifespan for people who have severe or advanced chronic lung conditions that do not respond to other treatments.

• #1 Interstitial lung disease – Wikipedia

  https://en.wikipedia.org/wiki/Interstitial_lung_disease

  ILD is not a single disease but encompasses many different pathological processes, hence treatment is different for each disease. If a specific occupational exposure cause is found, the person should avoid that environment. If a drug cause is suspected, that drug should be discontinued. […] Oxygen therapy at home is recommended in those with significantly low oxygen levels. Oxygen therapy in ILD is associated with improvements in quality of life but reductions in mortality are uncertain. Long-term oxygen therapy can be beneficial to people with ILD and hypoxemia to enhance gas exchange, lessen dyspnea, and increase physical activity. […] Pulmonary rehabilitation appears to be useful with the benefits being sustainable longer term with improvements in exercise capacity (as measured by a six minute walking test), dyspnea, and quality of life.

• #1 Treatments for Interstitial Lung Disease | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/healthcare-professionals/ild/treatments-ild

  Patients who have completed a Pulmonary Rehabilitation (PR) course experience less breathlessness and less fatigue. […] Patients with ILD typically suffer severe breathlessness. […] As the disease progresses, patients with ILDs often require long-term oxygen therapy (LTOT) due to chronic hypoxemia and to avoid complications such as pulmonary hypertension, right-sided heart failure (cor pulmonale), and polycythemia. […] Cough is a major symptom of ILDs. […] Understanding and managing cough in pulmonary fibrosis is essential for improving patients quality of life. […] For a select few patients, Lung transplantation may be considered if pulmonary fibrosis progresses and remains unresponsive to medical therapy.

• #1 Interstitial lung disease: Causes, outlook, and diagnosis

  https://www.medicalnewstoday.com/articles/320650

  There are treatment options that can help with management of the symptoms, however. Treatment also includes lifestyle changes to slow progression, as much as possible. […] There are currently no treatments that can completely reverse lung damage. But some treatments that are currently available may slow the progression of scarring and allow a person to breathe more freely. […] Many doctors recommend going through pulmonary rehabilitation, as this may help strengthen the lungs. Pulmonary rehabilitation uses various exercises to encourage a person to stretch their lung capacity and breathe better. […] Oxygen therapy may be prescribed to help reduce how breathless a person feels and to improve their ability to be active. However, a recent review of the research questions the usefulness of oxygen therapy long-term in those with interstitial lung disease.

• #1 Finding Relief for Interstitial Lung Disease | Blog | Loyola Medicine

  https://www.loyolamedicine.org/newsroom/blog-articles/interstitial-lung-disease-relief-treatments

  Lung transplant is a lifesaving surgery, says Dr. Dilling. […] Patients often ask me about the value of stem cell therapy, says Dr. Dilling. Dr. Glassberg’s work has shown us that mesenchymal stem cells are not an effective treatment, though research is ongoing. […] For patients with idiopathic pulmonary fibrosis, we may not be able to turn things around, but we can stabilize them and give them a better prognosis. […] Loyola’s pulmonology and critical care specialists also offer: Pulmonary rehabilitation to help patients improve their lung function, Palliative care to help patients take control of a life-limiting illness.

• #1 Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD) Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1343513-treatment

  Lung transplantation is the only available treatment for refractory connective tissue disease (CTD)-associated interstitial lung disease (ILD) (CTD-ILD) related with progressive fibrosis. […] In patients with disease refractory to treatment or who have extensive fibrosis, it is recommended that patients are referred to a transplant center for evaluation.

• #1 Interstitial Lung Disease Program

  https://www.massgeneral.org/medicine/pulmonary/treatments-and-services/interstitial-lung-disease

  We look at factors such as age, overall health, medical history, tolerance for certain therapeutic options, personal preferences and your test results to determine your individualized treatment plan. […] The interdisciplinary nature of our team offers many benefits for patients. We have the expertise to diagnose and treat the various forms of interstitial lung diseases and to address the complications of these disorders (e.g. sleep apnea, pulmonary hypertension) that can greatly compromise quality of life. […] Physicians and scientists in our program are working toward new advances for interstitial lung diseases for which current treatment options are limited. […] In addition to delivering exceptional clinical care, we offer patients the opportunity to participate in clinical trials of new therapies and scientific investigations into the causes of interstitial lung diseases.

• #1 Finding Relief for Interstitial Lung Disease | Blog | Loyola Medicine

  https://www.loyolamedicine.org/newsroom/blog-articles/interstitial-lung-disease-relief-treatments

  We cannot cure ILD yet, says pulmonologist and Loyola Medicine medical director of lung transplantation Daniel Dilling, MD. […] But we can provide relief from symptoms and slow the progress of the disease over time. […] Treatment of ILD, especially progressive forms such as idiopathic pulmonary fibrosis (IPF), has evolved over the past several decades. […] Since then, two antifibrotic medications (nintedanib and pirfenidone) have emerged as effective treatments for slowing IPF progression. […] Now, if I have a patient with a progressive ILD that isn’t IPF, I can prescribe nintedanib, says Dr. Dilling. […] We are part of the MILED clinical trial, investigating low-dose sirolimus therapy for people with mild disease, says Dr. Dilling. […] When ILD treatments are no longer effective, lung transplant may be an option.

• #1 Pharmacological Treatment of Interstitial Lung Diseases: A Novel Landscape for Inhaled Agents

  https://www.mdpi.com/1999-4923/16/11/1391

  The application of nanomedicine, which refers to the use of nanoscale particles as either active molecules or drug delivery vehicles in pharmaceutical formulations, to the treatment of ILDs has been investigated recently. Potentially delivered with DPIs and pMDIs as well as nebulizers, nanoparticles (NPs) may overcome some established aerosol therapy drawbacks. […] Various nanoparticle strategies have been studied and developed for the treatment of lung fibrosis; for example, Wang et al. used polymers, in particular poly (lactic-co-glycolic acid), as a delivery system for nintedanib, while Han et al. prepared lipid nanoparticles (Lip@VP) loaded with verteporfin (VER) and pirfenidone. […] Nintedanib is an indolinone derivative that was originally designed as an anti-angiogenic drug targeting the receptor tyrosine kinases VEGFR, FGFR, and PDGFR for the treatment of cancer. Its approval as an antifibrotic treatment in both IPF and PF-ILDs relies on its capability to interfere with lung fibroblast proliferation and migration as well as myofibroblast transformation. […] The efficacy of inhaled nintedanib has been investigated recently.

• #1

  https://www.pneumon.org/What-is-new-in-the-treatment-of-interstitial-lung-ndiseases,161867,0,2.html

  Lung transplantation remains an intervention that increases life expectancy, thus early referral of rapidly progressors or non-responders to available pharmacotherapies is advised. […] The main challenge of trials of novel compounds is the fact that they should be effective as an add-on therapy, given that novel agents cannot be compared to true placebo. […] The extended-release form of the dual-acting opioid receptor agonist/ opioid receptor antagonist, nalbuphine, has great potential with regard to chronic cough reduction in patients with pulmonary fibrosis.

• #1

  https://www.pneumon.org/What-is-new-in-the-treatment-of-interstitial-lung-ndiseases,161867,0,2.html

  In cases of SSc with life-threatening organ involvement, autologous hematopoietic stem cell transplantation is recommended, only in an individualized benefit and risk assessment context. […] The cornerstone of HP treatment is complete avoidance of the causative agent, if identified, in order to prevent disease progression. […] Fibrotic HP is more likely to respond to antifibrotics and nintedanib has recently been shown to be beneficial in HP when presenting as PPF. […] According to the results of the INBUILD trial and the new guidelines, treatment with nintedanib is suggested in PFF, when conventional therapies have failed. […] Comprehensive ILD management extends beyond pharmacological therapy, as non-pharmacological interventions are also essential. […] Given that ILDs are often associated with dramatic restraints in everyday life and survival, palliative care consultation should also be considered timely, with patient-centered interventions.

• #1 Interstitial Lung Disease Treatment Options | Temple Health

  https://www.templehealth.org/services/conditions/interstitial-lung-diseases/treatment-options

  If you smoke, the most important thing you can do is quit smoking. Your doctor can help you by recommending smoking cessation programs and products. […] In addition, your doctor may recommend pulmonary rehabilitation, which encompasses a variety of methods designed to improve the well-being of people with chronic breathing problems. […] Depending on the underlying cause of your ILD, your doctor may recommend different medications. Some of the medications used to treat ILD include: […] Inhaled or oral corticosteroid medications can reduce the immune system activity that causes scarring and inflammation in the lungs in some forms of ILD. […] Antibiotics treat these infections. […] Disease-specific therapies such as medicines specifically for pulmonary fibrosis. […] Immunomodulators therapies that alter the way the immune system functions.

• #1 Interstitial Lung Disease (Interstitial Pneumonia): Symptoms, Treatment & Types

  https://www.emedicinehealth.com/interstitial_lung_disease/article_em.htm

  Lung Exam Interstitial lung disease includes a group of diseases that have thickening of the supporting tissues between the air sacs of the lungs as the common factor. […] Treatment depends on the underlying cause of the inflammation. […] Corticosteroid drugs and immune-suppressing drugs may be used in the management of interstitial lung disease. […] Smoking cessation is essential for people with interstitial lung disease. […] The choice of treatment depends upon a number of factors including the cause of the interstitial lung disease and the overall health status of the patient. […] Most commonly, corticosteroid drugs are given in an attempt to reduce inflammation. […] Sometimes, immune-suppressing drugs, such as azathioprine (Imuran) or cyclophosphamide (Cytoxan) are also given, either in combination with steroids or following a course of steroid treatment.

• #1 Interstitial Lung Disease | Boston Children’s Hospital

  https://www.childrenshospital.org/conditions/interstitial-lung-disease

  Reducing the excess work of breathing: It is important to reduce the work required to breathe so that your child can use more of the calories required to grow. […] Providing nutritional support as needed to promote growth: Children with ILD may work harder to breathe and may find it difficult to take in enough calories to supply the energy to breathe and grow. […] Preventing further damage to the lungs from the underlying condition and associated conditions: Infections can cause a flare-up in respiratory symptoms in children with lung diseases, so we recommend that you keep your child’s vaccinations up to date. […] Sometimes lung transplantation becomes a treatment option for children with ILD. If this is the case for your child, the Lung Transplant Program team here at Boston Children’s Hospital will provide expert review and opinion, as well as other services and support.

• #1 Interstitial lung disease – Wikipedia

  https://en.wikipedia.org/wiki/Interstitial_lung_disease

  The immunomodulator tocilizumab has a benefit in scleroderma associated ILD by helping to preserve lung function (as measured by FVC) at 48 weeks. The immunomodulators cyclophosphamide, mycophenolate mofetil and rituximab all showed improved lung function (as measured by % predicted FVC) compared to placebo in systemic sclerosis or scleroderma associated ILD. […] The inhaled vasodilator treprostinil (a synthetic prostacyclin which acts as a prostaglandin I2 analogue) is indicated in the treatment of pulmonary hypertension secondary to interstitial lung disease and is associated with improved exercise capacity as measured by a 6-minute walk test. […] Those with ILD should stop smoking cigarettes if they smoke. Vaccinations against pneumococcus, Covid-19, RSV and influenza are indicated in all those with ILD. Short acting opiates are known to improve breathlessness symptoms in those with end stage lung disease. The opiate agonist-antagonist nalbuphine and morphine are also known to improve coughing in those with ILD and other end stage lung diseases.

• #1 Management of Interstitial Lung Disease: Emerging Therapies and Unmet Needs

  https://www.ajmc.com/view/management-of-interstitial-lung-disease-emerging-therapies-and-unmet-needs

  Nintedanib and pirfenidone are treatments approved for use in patients with IPF. […] In the phase 3 INPULSIS-1 and INPULSIS-2 trials, patients with IPF who received nintedanib had a significantly lower adjusted annual rate of change in FVC compared with those given placebo. […] PF-ILD has similar clinical and pathophysiologic features to IPF, and it was recently proposed that antifibrotic treatments used for IPF could be effective for PF-ILD. […] In 2020, nintedanib became the first FDA-approved treatment for ILDs that have a progressive phenotype (ie, those caused by autoimmune conditions, hypersensitivity pneumonitis, and idiopathic NSIP). […] Cyclophosphamide was one of the initial immunosuppressive therapies that showed promise for SSc-ILD based on outcomes from the Scleroderma Lung Study I, which compared 1 year of cyclophosphamide therapy with placebo use.

• #1 Nonspecific Interstitial Pneumonia: Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/14804-nonspecific-interstitial-pneumonia-nsip

  Nonspecific interstitial pneumonia (NSIP) is a type of interstitial lung disease that causes inflammation between the air sacs of your lungs. […] If you dont have scarring in your lungs, NSIP is usually curable with treatment. […] Providers treat nonspecific interstitial pneumonia with medications that reduce inflammation and manage underlying conditions. In severe cases, treatments like pulmonary rehabilitation and oxygen therapy can strengthen your lungs and help you get more oxygen. If your condition worsens despite treatment, a lung transplant may be an option. […] Specific medications and procedures to treat NSIP include: Prednisone or other corticosteroids. Immunosuppressive and cytotoxic drugs, including azathioprine, cyclophosphamide and mycophenolate. Anti-fibrotic drugs, including pirfenidone and nintedanib. Rituximab, a biologic drug for connective tissue disorders. Pulmonary rehabilitation. Oxygen therapy. Lung transplant. […] Many people with NSIP get better with treatment and avoid permanent lung damage.

• #1 Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD) Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/1343513-treatment

  In patients with suspected connective tissue disease (CTD)-associated interstitial lung disease (ILD) (CTD-ILD), referral to a center with expertise in management of CTD-ILD is recommended. A multidisciplinary approach to help guide management includes collaboration with pulmonologists, rheumatologists, radiologists, and pathologists. Furthermore, referral to centers with expertise in pulmonary hypertension on lung transplantation may be required, depending on the individual clinical context. […] The treatment of CTD-ILD requires immunosuppression with either steroids or steroid-sparing agents. […] Lung transplantation may be an option for those with end-stage lung disease. […] The mainstay of therapy requires immunosuppression either through steroids or steroid-sparing agents. […] Prolonged treatment with corticosteroids leads to a large number of comorbidities such as diabetes mellitus, hypertension, osteoporosis, and psychiatric disease. Therefore, all efforts should be made to decrease the steroid burden as soon as possible.

• #1 Management of Interstitial Lung Disease: Emerging Therapies and Unmet Needs

  https://www.ajmc.com/view/management-of-interstitial-lung-disease-emerging-therapies-and-unmet-needs

  Mycophenolate mofetil (MMF), which has a relatively favorable safety profile and provides effective immunosuppression in patients with SSc-ILD, was compared with cyclophosphamide in the Scleroderma Lung Study II. […] SSc-ILD and IPF share some pathophysiologic mechanisms; therefore, nintedanib was believed to be a promising therapeutic option to address the progressive fibrosis component. […] Consideration of an antifibrotic agent such as nintedanib or pirfenidone is important if the patient is suspected to have IPF. […] However, agents targeting the immune system (eg, steroids and steroid-sparing agents that include MMF) often can prevent progression and delay the need for antifibrotic agents in patients with ILD caused by hypersensitivity pneumonitis or a connective tissue disorder. […] Many of the newer agents used for ILD (eg, nintedanib, pirfenidone, and tocilizumab) have a higher financial cost.

• #1 Pharmacological Treatment of Interstitial Lung Diseases: A Novel Landscape for Inhaled Agents

  https://www.mdpi.com/1999-4923/16/11/1391

  Interstitial lung diseases (ILDs) encompass a heterogeneous group of over 200 disorders that require individualized treatment. Antifibrotic agents, such as nintedanib and pirfenidone, have remarkably revolutionized the treatment landscape of patients with idiopathic pulmonary fibrosis (IPF). […] The intent of this paper is to provide a broad perspective of the current state of the art and the development of inhalation-based agents in the treatment of ILDs that would offer the opportunity to tackle airway dysfunction, limit fibrogenetic processes, and improve endothelial dysfunction and pulmonary vasculature remodeling with a potentially more favorable safety profile. […] Pirfenidone, an antifibrotic and anti-inflammatory agent capable of inhibiting fibroblast proliferation and collagen deposition by targeting the TGF-β pathway, has gained approval for the treatment of IPF with a recommended full oral dosage of 2403 mg/day divided into three doses. Nintedanib is an intracellular multitarget tyrosine kinase inhibitor that is licensed not only for the treatment of IPF but also for other conditions. Nintedanib has also gained approval for PF-ILDs other than IPF as well as for systemic sclerosis–ILD (SSc-ILD). […] The decision should instead be the result of a tailored approach in which clinician–patient cooperation is crucial in reducing adverse effects and optimizing patient compliance.

• #1 Interstitial Lung Disease Program | Temple Health

  https://www.templehealth.org/services/lung/patient-care/programs/interstitial-lung-disease

  Interstitial lung disease (ILD) is an umbrella term for disorders involving scarring or inflammation of the tissue surrounding the air sacs in the lungs. Since many patients with interstitial lung disease get worse over time, getting a diagnosis and beginning treatment as soon as possible is important. […] We offer a high standard of treatment for patients with interstitial lung disease. Our specialists are experienced in prescribing and managing drug therapy, including nintedanib and pirfenidone. These drugs are FDA approved for the treatment of IPF but can be difficult to manage. For this reason, it’s important for treatment with these drugs to be overseen by physicians who are experienced in their use. […] In patients with advanced disease, lung transplant is an important treatment option. We work closely with Temples Lung Transplant Program to provide patients with one of the best transplant outcomes in the Tri-State area.

• #1 Interstitial Lung Disease – Advanced Lung Disease Center | Loma Linda University Health

  https://lluh.org/services/advanced-lung-disease-center/our-services/interstitial-lung-disease

  We offer a wide range of treatment options, including anti-inflammatory agents (to reduce inflammation), anti-fibrotic therapies (to prevent or reduce lung tissue scarring) and biologics (to suppress the immune response causing the inflammation). […] The clinical course of ILD can be unpredictable. We closely monitor your symptoms and lung function, then modify your treatment plan as needed to optimize your care.

• #1 Interstitial Lung Disease Clinic | Baylor Medicine

  https://www.bcm.edu/healthcare/specialties/the-lung-institute/pulmonary-medicine/interstitial-lung-disease-clinic

  Baylor Medicines Interstitial Lung Disease (ILD) program offers specialized and multidisciplinary care. […] Our collaborative nature ensures you have an individualized treatment plan that considers all aspects of your health. […] In addition, our physicians are at the forefront of medical research and innovation, allowing patients access to state-of-the-art treatment modalities and the latest clinical research trials. […] The ILD team works in close collaboration with the Baylor Medicine Lung Transplant program, which has one of the highest numbers of cases in the United States. […] Patients will benefit from being seen at specialized centers that require close monitoring of specialized medications. Some of the patients may require lung transplantation.

• #2 Interstitial Lung Disease Treatment Options | Temple Health

  https://www.templehealth.org/services/conditions/interstitial-lung-diseases/treatment-options

  Interstitial Lung Disease Treatment Options video with Dr. Erin Camac Temple pulmonologists know the importance of making an accurate diagnosis when it comes to interstitial lung diseases and are able to offer a variety of treatment options. […] Usually, the scarring of the lungs that occurs with interstitial lung disease (ILD) is not reversible. But treatments that improve lung function are available for diseases which include active inflammation. […] The purpose of interstitial lung disease treatment is usually to: […] Slow or stop the progression of the disease […] Improve quality of life […] In some cases, reverse the effects of ILD. […] Treatment may vary depending on the underlying cause. Temple interstitial lung disease specialists offer the following treatments that may be used alone or in combination:

• #2 Interstitial Lung Disease | Condition | UT Southwestern Medical Center

  https://utswmed.org/conditions-treatments/interstitial-lung-disease/

  UT Southwestern Medical Center brings together skilled specialists from several disciplines to provide high-quality care for people who have interstitial lung disease and pulmonary fibrosis. Our team-based approach to care offers patients the expertise and advanced treatment options they need to relieve symptoms and improve their quality of life. […] The care team works to develop an individualized treatment plan based on each patients overall health, specific symptoms, and other health conditions. Treatment plans can include: […] Medications to treat ILD and its symptoms include: Pirfenidone (Esbriet) and nintedanib (OFEV) to slow progression of the disease. […] Our nonsurgical therapies include oxygen therapy, which can ease breathing, reduce the risk of complications from low blood-oxygen levels, and prevent high blood pressure in the lungs (pulmonary hypertension). […] For people with advanced PF and severe lung damage, a single or double lung transplant can improve the ability to breathe and exercise.

• #2 Basics of Interstitial Lung Diseases | ILD Collaborative

  https://www.ildcollaborative.org/resources/basics-of-ilds

  An ILD that primarily causes fibrosis (scarring) in the interstitium can be treated with anti-fibrotic medications. For example, idiopathic pulmonary fibrosis (IPF) causes fibrosis (scarring) and can be treated with nintedanib or pirfenidone. A person with an ILD that primarily causes fibrosis will not be helped by anti-inflammatory drugs or immune suppressant drugs.

• #2 Interstitial Lung Disease (ILD) Treatment | Froedtert & MCW

  https://www.froedtert.com/interstitial-lung-disease

  Although it does not currently have a cure, ILD is treated based on the kind of lung disease you have and other co-morbidities that might exist. We tailor the therapy for your ILD based on the extent/severity of your lung disease, medical history, age, and your personal preferences for your health care. Examples of available therapies include: […] Corticosteroids: Oral corticosteroids (i.e. Prednisone) are the mainstay of treatment for many ILDs. They are used as mono-therapy or can be used in conjunction with other steroid-sparing agents. […] Steroid sparing agents: These are medications that tone-down your immune system, decreasing inflammation and scarring in the lungs. Common medications may include Azathioprine, Mycophenolate Mofetil and Methotrexate. […] Anti-fibrotic medications: A class of medications that slow (or, sometimes, halt) scarring (fibrosis). These include Pirfenidone and Nintedanib. […] Investigative medications: We participate in clinical trials evaluating the use of new medications in ILD. […] Oxygen therapy […] Pulmonary rehabilitation […] Smoking cessation […] Diagnosing and treating acid reflux (heart burn) […] Lung transplant.

• #2

  https://www.pulmonaryfibrosis.org/understanding-pff/treatment-options/medications

  Tocilizumab is a biologic medication that is used to prevent and treat inflammation by interrupting the inflammatory process. It has been shown to reduce the rate of decline in pulmonary function in adult patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). […] Prednisone is used to treat and prevent inflammation by weakening the immune system. While prednisone is not usually used to treat idiopathic pulmonary fibrosis, it is sometimes used to treat inflammation in the lungs of people living with other forms of pulmonary fibrosis. […] Similar to prednisone, mycophenolate mofetil (MMF) also can treat and prevent inflammation by suppressing the immune system. MMF is sometimes used in combination with prednisone, but is also used alone. […] Azathioprine is used to suppress the immune system similarly to MMF. Several other anti-inflammatory therapies such as methotrexate, cyclophosphamide, cyclosporine, rapamycin (sirolimus), and tacrolimus have been used to treat different forms of interstitial lung disease. As mentioned previously, treatment with these agents is often tailored to specific diseases or forms of ILD and pulmonary fibrosis.

• #2 Management – Lung Foundation Australia

  https://lungfoundation.com.au/health-professionals/conditions/interstitial-lung-disease/management/

  High doses of prednisolone (including intravenous methylprednisolone) may be used in conditions which are predominantly inflammatory, in which case, improvement is the aim of treatment. […] After the initial phase, the aim of immunosuppressive treatment is to halt progression and stabilise disease. […] Anti-fibrotic therapy is currently recommended in mild-to-moderate IPF and PPF; Current anti-fibrotic therapies (Nintedanib (OfevTM) and Pirfenidone (EsbrietTM)) are aimed at slowing disease progression. […] Nintedanib or Pirfenidone are recommended in the treatment of mild-to-moderate Idiopathic Pulmonary Fibrosis (IPF), whereas only Nintedanib is recommended in the treatment of PPF. […] In general, a patient on antifibrotic therapy would lose a mean of about 120ml of lung function a year, compared to someone not on treatment who would lose about 240ml of lung function a year.

• #2 Interstitial Lung Disease: Stages, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17809-interstitial-lung-disease

  Oxygen therapy. Your provider will prescribe extra oxygen if you dont have enough getting to your blood or tissues. Its delivered through a mask or tube in your nose. […] Lung transplant. Some people with severe cases of ILD get a lung transplant. […] Some treatments for interstitial lung disease can weaken your immune system. Your provider will monitor you closely for complications if youre taking one of these medications.

• #2 Pharmacological Treatment of Interstitial Lung Diseases: A Novel Landscape for Inhaled Agents

  https://www.mdpi.com/1999-4923/16/11/1391

  Similarly, inhaled pirfenidone has been tested in early studies; in the ATLAS study, a phase 1b trial, the safety and efficacy of inhaled pirfenidone (AP01) at a dose of 50 mg once per day or 100 mg two times per day were assessed in IPF patients who were intolerant, reluctant, or ineligible for oral pirfenidone or nintedanib. […] Alongside a potential reduction in adverse events, inhalation of antifibrotic agents can result in more effective antifibrotic properties possibly slowing the pace of FVC decline. […] Treprostinil, a prostacyclin analog, may be considered in patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD) based on the findings from the INCREASE study. […] The phase 3 trial evaluating the efficacy of inhaled treprostinil in patients with IPF is ongoing; results are expected by 2025. […] Remarkable progress has been achieved in the treatment of ILDs. […] Investigation of novel molecules and formulations is currently ongoing in new recruiting trials.

• #2

  https://www.nhs.uk/conditions/idiopathic-pulmonary-fibrosis/treatment/

  There’s currently no cure for idiopathic pulmonary fibrosis (IPF). The main aim of treatment is to relieve the symptoms as much as possible and slow down its progression. […] There are 2 medicines that can help slow down the progression of IPF in some people: pirfenidone and nintedanib. […] Pirfenidone has been shown to help slow down the process of scarring in the lungs by reducing the activity of the immune system. […] Nintedanib is a newer medicine that can also help slow down scarring of the lungs in some people with IPF. […] If this happens, oxygen treatment can help with your breathing and allow you to be more active. […] Pulmonary rehabilitation is used for many long-term lung conditions. […] If your condition continues to get worse despite treatment, your specialist may recommend a lung transplant. […] If you’re told there’s nothing more that can be done to treat you, or you decide not to have treatment, your GP or care team will give you support and treatment to relieve your symptoms. This is called palliative care.

• #2 Interstitial Lung Disease: Stages, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17809-interstitial-lung-disease

  Theres no cure for interstitial lung disease. Treatment for ILD usually focuses on treating underlying disease and improving your symptoms. Your healthcare provider might prescribe physical therapy, supplemental oxygen or medication to reduce inflammation or slow down the disease. […] Corticosteroids. Drugs like prednisone can help reduce inflammation. […] Anti-fibrotic and cytotoxic drugs. These medications can slow down lung scarring. They include azathioprine, cyclophosphamide, pirfenidone and nintedanib. […] Biologic drugs. Medications like rituximab are sometimes used to treat autoimmune diseases and other causes of ILD. […] Treatment for GERD. Gastroesophageal reflux disease (GERD) can make ILD worse, so your provider may prescribe medications to keep stomach acid down. […] Pulmonary rehabilitation. Breathing exercises and physical therapy can make your lungs stronger and breathing easier.

• #2 Interstitial Lung Disease Program

  https://www.massgeneral.org/medicine/pulmonary/treatments-and-services/interstitial-lung-disease

  We look at factors such as age, overall health, medical history, tolerance for certain therapeutic options, personal preferences and your test results to determine your individualized treatment plan. […] The interdisciplinary nature of our team offers many benefits for patients. We have the expertise to diagnose and treat the various forms of interstitial lung diseases and to address the complications of these disorders (e.g. sleep apnea, pulmonary hypertension) that can greatly compromise quality of life. […] Physicians and scientists in our program are working toward new advances for interstitial lung diseases for which current treatment options are limited. […] In addition to delivering exceptional clinical care, we offer patients the opportunity to participate in clinical trials of new therapies and scientific investigations into the causes of interstitial lung diseases.

• #3 Interstitial Lung Diseases – Treatment | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/interstitial-lung-diseases/treatment

  Your treatment for ILDs will depend on the cause of your condition and how serious your symptoms are. Your healthcare provider will work with you to decide on a treatment plan for you. […] Treatment for ILDs does not cure your lung damage, but it can prevent lung damage from getting worse and can help you breathe easier. […] Depending on the type of ILD you have, your doctor may prescribe medicine to help you breathe easier. […] Oxygen therapy is a treatment that delivers oxygen gas for you to breathe. […] Oxygen therapy can be given for a short or long period of time in the hospital, another medical setting, or at home. […] Pulmonary rehabilitation is a supervised program that includes exercise training, health education, and breathing techniques for people who have certain lung conditions, lung problems due to other conditions, or after a lung transplant. […] Lung transplant is surgery to remove a diseased lung and replace it with a healthy lung. […] Lung transplants are used to improve quality of life and extend the lifespan for people who have severe or advanced chronic lung conditions that do not respond to other treatments.

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