Materiały źródłowe

• #1 Pulmonary fibrosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/diagnosis-treatment/drc-20353695

  The lung scarring and thickening that occurs in pulmonary fibrosis cannot be repaired. And no current treatment has proved effective in stopping the disease from getting worse over time. Some treatments may improve symptoms for a time or slow how fast the disease worsens. Others may help improve quality of life. […] Treatment depends on the cause of your pulmonary fibrosis. Doctors and other healthcare professionals evaluate how severe your condition is. Then together you can decide on the best treatment plan. […] If you have idiopathic pulmonary fibrosis, your healthcare professional may recommend the medicine pirfenidone (Esbriet) or nintedanib (Ofev). Both are approved by the U.S. Food and Drug Administration (FDA) for idiopathic pulmonary fibrosis. Nintedanib also is approved for other types of pulmonary fibrosis that get worse quickly. These medicines may help slow the worsening of pulmonary fibrosis and may prevent bouts when symptoms suddenly get worse.

• #2 How Is Pulmonary Fibrosis Treated? | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/how-is-pulmonary-fibrosis-treated

  There is no cure for pulmonary fibrosis. Current treatments are aimed at slowing the course of the disease, relieving symptoms and helping you stay active and healthy. Treatments for PF include: […] Depending on the type of pulmonary fibrosis you have, there may be medications to slow progression of the disease and others that will help relieve your symptoms. Only your medical provider can determine if any medications may be beneficial for you. […] Oxygen therapy (sometimes called supplemental oxygen) may be prescribed if your lung disease is preventing a healthy level of oxygen from getting into your bloodstream. It may help reduce your shortness of breath and make it easier for you to stay active. […] Pulmonary rehabilitation is a program that teaches you about your lung disease, how to exercise and manage your disease and provides support and counseling.

• #3 Pulmonary Fibrosis Treatment Options | Temple Health

  https://www.templehealth.org/services/conditions/pulmonary-fibrosis/treatment-options

  Treatment of pulmonary fibrosis focuses on alleviating symptoms and improving quality of life. Unfortunately, the scarring of the lungs that occurs with pulmonary fibrosis (PF) cannot be reversed. But with early and accurate diagnosis, targeted treatments supplemented with oxygen therapy and pulmonary rehabilitation may help improve breathing and slow the progress of the disease. […] Together, our specialists work closely with your doctor to find out the cause and severity of your pulmonary fibrosis. Using this information, Temples pulmonary fibrosis team will develop a treatment plan tailored to your needs. This may include a combination of lifestyle changes, medications, supplemental oxygen, and pulmonary rehab. If your pulmonary fibrosis is very advanced, your team may recommend a lung transplant through Temples Lung Transplant Program, one of the most active programs in the country.

• #4

  https://link.springer.com/article/10.1007/s41030-023-00216-0

  Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial lung disease of unknown aetiology. […] Two anti-fibrotic medications (nintedanib and pirfenidone) have been approved for the treatment of IPF. […] Early diagnosis is crucial to ensure timely treatment selection and improve outcomes. […] Anti-fibrotic medications slow the decline in lung function in patients with IPF. […] There is a growing body of evidence suggesting that anti-fibrotic medications reduce the risk of acute deteriorations in lung function and improve life expectancy in IPF. […] Early diagnosis of IPF is crucial to ensure timely treatment selection and improve outcomes. […] Treatment access could be improved by education of non-respiratory clinicians about the presenting symptoms of IPF, utilising computer-aided informatics, streamlining referral pathways and planned changes to the %FVC requirement for people to start anti-fibrotic medications.

• #5 Pulmonary Fibrosis Medications | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/how-is-pulmonary-fibrosis-treated/medications

  Depending on the type of pulmonary fibrosis you have, there may be medicine available to treat your disease. Only your medical provider can determine if these medications may be beneficial for you. […] Currently, two drugs are FDA-approved for treatment of idiopathic pulmonary fibrosis (IPF), which is the most common form of PF. These include nintedanib (Ofev) and pirfenidone (Esbriet). These medications are called anti-fibrotic agents, meaning that they have shown in clinical trials to slow down the rate of fibrosis or scarring in the lungs. […] While taking these medications, you will be monitored closely for any serious side effects. Monitoring will include routine blood work to make sure your liver remains healthy. […] There are certain types of PF (excluding IPF) that respond to treatment with steroids. Steroids, also known as glucocorticoids, are a class of medication that reduces inflammation (swelling).

• #6

  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. […] 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.

• #7 Idiopathic Pulmonary Fibrosis (IPF) Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/301226-treatment

  Treatment considerations should include both pharmacological and nonpharmacological interventions including oxygen supplementation (if hypoxemic) and/or pulmonary rehabilitation. Any patient with idiopathic pulmonary fibrosis who is a current smoker should be encouraged to quit and offered pharmacologic therapy if needed. Palliative care may help with symptom management (cough, dyspnea, and/or anxiety). Acute exacerbations should be treated with corticosteroids […] Vaccination against COVID 19, RSV, influenza and pneumococcal infection should be encouraged in all patients with idiopathic pulmonary fibrosis. […] Current guidelines recommend against the use of antacid therapy to treat idiopathic pulmonary fibrosis for the purpose of improving respiratory outcomes. […] Nintedanib is a tyrosine kinase inhibitor that was initially developed as an anti-tumor agent before it was noted to have activity against fibroblasts through inhibition of vascular endothelial growth factor (VEGF) and other profibrotic mediators such as platelet-derived growth factor (PDGF), fibroblast growth factor (FGF), and transforming growth factor (TGF).

• #8 Idiopathic Pulmonary Fibrosis (IPF) Medication: Tyrosine Kinase Inhibitors, Antifibrotic Agents

  https://emedicine.medscape.com/article/301226-medication

  Ziritaxestat, also known as GLPG-1690, which targets autotaxin, an enzyme responsible for lysophosphatidic acid production, was also examined in a phase 2 trial in 2018 and may reduce circulating lysophosphatidic acid levels while influencing lung function and findings on functional respiratory imaging. […] The subsequent phase 3 trials, ISABELA 1 and ISABELA 2, enrolled 1306 participants suffering from IPF treated with GLPG1690 600 or 200mg (in addition to local standard of care) or placebo for at least 52weeks. […] Nintedanib inhibits multiple tyrosine kinases and targets growth factors, which have been shown to be potentially involved in pulmonary fibrosis (eg, vascular endothelial growth factor receptor [VEGFR], fibroblast growth factor receptor [FGFR], platelet-derived growth factor receptor [PDGF]).

• #9 Guidelines for the Medical Treatment of Idiopathic Pulmonary Fibrosis | Archivos de Bronconeumología

  https://www.archbronconeumol.org/en-guidelines-for-medical-treatment-idiopathic-articulo-S1579212917300782

  Pirfenidone is a pleiotropic drug with antifibrotic and antiinflammatory properties, which inhibits the synthesis of profibrinogenic growth factors, such as transforming growth factor-beta (TGF-) and platelet-derived growth factor (PDGF), fibroblast proliferation, and formation of collagen. Its clinical efficacy has been evaluated in several double-blind, placebo-controlled randomized clinical trials: 2 Asian studies, 1 phase II and 1 phase III, and 3 phase III studies in the West (CAPACITY-1, CAPACITY-2, and ASCEND). […] The recommended dose of pirfenidone is 2403mg/day divided in 3 doses to be taken with meals. The maximum dose is reached after 3 weeks of progressive dose escalation: in week 1, one 267mg tablet is administered every 8h, in week 2, two 267mg tablets every 8h and in week 3, three 267mg tablets every 8h. The most significant adverse events are gastrointestinal (nausea, dyspepsia, anorexia, and weight loss), followed by rash, photosensitivity, and to a lesser extent, liver function changes.

• #10 Idiopathic Pulmonary Fibrosis (IPF) Medication: Tyrosine Kinase Inhibitors, Antifibrotic Agents

  https://emedicine.medscape.com/article/301226-medication

  The precise mechanism by which pirfenidone may work in pulmonary fibrosis has not been established. It inhibits transforming growth factor (TGF)-, a chemical mediator that controls many cell functions, including proliferation and differentiation. It also inhibits the synthesis of TNF-, a cytokine that is known to have an active role in inflammation.

• #11 Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-019-1161-4

  Clinical trials have demonstrated that nintedanib and pirfenidone reduce the decline in lung function in patients with IPF, with consistent effects across the spectrum of baseline FVC studied. […] Although individual clinical trials have not been powered to show significant effects on acute exacerbations and mortality, there is a growing body of evidence that nintedanib and pirfenidone reduce the risk of acute deteriorations in lung function and improve life expectancy by reducing the rate at which IPF progresses. […] Prompt treatment of IPF is critical to preserving patients lung function, reducing the risk of acute exacerbations and improving outcomes. […] Physicians have a key role to play in explaining to patients that the aim of drug therapy is to slow the progression of their disease and that decline in FVC while taking a drug may not indicate a failure of treatment.

• #12 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.

• #13 Pulmonary Fibrosis Medications | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/how-is-pulmonary-fibrosis-treated/medications

  Other groups of medications prescribed to reduce inflammation include azathioprine, cyclophosphamide and mycophenolate mofetil (Cellcept, Myfortic), which work by suppressing the immune system. […] Doctors may recommend anti-acid medications to treat gastroesophageal reflux disease (GERD), a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis. […] A dry, hacking cough is a common symptom of PF. Treating cough is mostly trial and error. What works for one patient may not work for another. Some treatment options for cough include: Over the counter cough medicine like Robitusson and cough drops, Prescription cough medicine like hydrocodone (Tussionex PennKenetic) and benzonatate (Tessalon Perles), Thalidomide (Thalomid) may be used for serious cases of cough in some patients.

• #14

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

  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. […] Treatment with these agents is often tailored to specific diseases or forms of ILD and pulmonary fibrosis.

• #15 Pulmonary fibrosis – Wikipedia

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

  Anti-inflammatory agents have only limited success in reducing the fibrotic process. Some other types of fibrosis, such as non-specific interstitial pneumonia, may respond to immunosuppressive therapy such as corticosteroids. But only a minority of patients respond to corticosteroids alone, so additional immunosuppressants, such as cyclophosphamide, azathioprine, methotrexate, penicillamine, and cyclosporine may be used. Colchicine has also been used with limited success. Trials with newer drugs such as IFN- and mycophenolate mofetil are ongoing. […] Hypersensitivity pneumonitis, a less severe form of pulmonary fibrosis, is prevented from becoming aggravated by avoiding contact with the causative material.

• #16

  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 not indicated for the treatment of IPF, but like prednisone can be used for people living with other forms of PF.

• #17 Pulmonary Fibrosis Causes, Symptoms, Stages & Life Expectancy

  https://www.medicinenet.com/pulmonary_fibrosis/article.htm

  The toxicity and side effects of treatments can be serious. Therefore, patients with pulmonary fibrosis should be followed by a lung specialist experienced in this condition. The lung specialist will determine the need and duration of treatment and will monitor the response to therapy along with any side effects. […] Only a minority of patients respond to corticosteroids alone, so other immune-suppressing medications are used in addition to corticosteroids especially if a different cause for interstitial lung disease is suspected. These include: cyclophosphamide (Cytoxan), azathioprine (Imuran, Azasan), methotrexate (Rheumatrex, Trexall), penicillamine (Cuprimine, Depen), cyclosporine. […] The anti-inflammatory medication colchicine has also been used with limited success. Other trials using drugs such as gamma interferon and mycophenolate mofetil (Cellcept) have not met with much success in the treatment of idiopathic pulmonary fibrosis.

• #18 Immunosuppressive therapies for pulmonary fibrosis | Action for Pulmonary Fibrosis

  https://www.actionpf.org/information-support/treatments-for-pulmonary-fibrosis

  Your treatment will depend on the type of pulmonary fibrosis or interstitial lung disease that you have. Because everyone is different, you will be able to discuss your medication with your medical team so together you can find the treatment that suits you best. […] Immunosuppressive therapies are used for certain conditions where inflammation in the lungs may also occur, such as hypersensitivity pneumonitis or autoimmune diseases including rheumatoid arthritis and systemic sclerosis. […] If your pulmonary fibrosis is caused by an autoimmune disease (such as rheumatoid arthritis, autoimmune myositis, Sjogrens syndrome or systemic sclerosis) or hypersensitivity pneumonitis, you may take an immunosuppressant to reduce inflammation in your lungs. […] Immunosuppressants used to treat these conditions include mycophenolate mofetil, cyclophosphamide, methotrexate, rituximab and azathioprine. […] You may also be offered other treatment such as medications to help manage a cough or acid reflux and advice to manage breathlessness.

• #19 Pulmonary Fibrosis – Treatment | NHLBI, NIH

  https://www.nhlbi.nih.gov/health/idiopathic-pulmonary-fibrosis/treatment

  There is currently no cure for IPF. You may need medicines, pulmonary rehabilitation, procedures, or other treatments to slow down lung damage and help improve your quality of life. […] You may need the following medicines: […] Nintedanib or pirfenidone can help your lungs work better. They may also help to prevent an acute exacerbation, which is an unexpected and sudden worsening of symptoms over a period of days or weeks, and may prolong survival. […] Antacids may help prevent stomach acid from getting into the lungs from reflux, making IPF worse. […] You may need other treatments to treat IPF, including: […] Oxygen therapy to decrease shortness of breath and improve the ability to exercise […] Ventilator support to help with breathing. […] A lung transplant may be an option for some people who have serious IPF. The major complications of a lung transplant are infection and rejection of the new organ by the body. You will have to take medicines for the rest of your life to reduce the risk of rejection following a lung transplant.

• #20 Pulmonary fibrosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pulmonary-fibrosis/diagnosis-treatment/drc-20353695

  New medicines and therapies are being developed or tested in clinical trials but are not yet approved by the Food and Drug Administration (FDA). Researchers continue to study medicines to treat pulmonary fibrosis. […] Using extra oxygen, called supplemental oxygen, cannot stop lung damage, but it can: Make breathing and exercise easier. Prevent or lessen complications from low blood oxygen levels. Possibly lessen strain on the right side of the heart. Improve sleep and sense of well-being. […] Pulmonary rehabilitation can help manage your symptoms and improve your ability to do daily tasks. […] A lung transplant may be an option for some people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. But a lung transplant can involve complications such as rejection and infection. After a lung transplant, you take medicines for the rest of your life. You and your healthcare team may discuss a lung transplant if it’s thought to be the right treatment option for your condition.

• #21

  https://umiamihealth.org/en/treatments-and-services/pulmonary-medicine/pulmonary-fibrosis

  Pulmonary fibrosis is scarring (called fibrosis) in the tissue inside the lungs. Its just one of about 200 interstitial pulmonary diseases that affect the tissue around the balloon-like structures (called air sacs) attached to the lung airways. […] If you have symptoms of pulmonary fibrosis, visit a pulmonologist (lung specialist) at the University of Miami Health System. Well manage your symptoms, so that you can live a healthier life. […] Medicine therapy Oral and injected medications can decrease inflammation, suppress an overactive immune system, and slow scarring progression. These include corticosteroids, chemotherapy, and immunotherapy drugs. […] Oxygen therapy If blood oxygen levels are too low, your doctor may prescribe inhaled oxygen as needed, such as before exercise or long-term for most of the day. There are different devices available. Many are portable, meaning theyre easy to move or take with you on the go.

• #22 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. […] 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.

• #23

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

  Pulmonary rehabilitation (PR) is a structured exercise program designed for people living with chronic lung diseases like pulmonary fibrosis (PF). […] Pulmonary rehabilitation has been found to improve physical function, breathlessness (dyspnea), mood, and quality of life in people with IPF and other types of PF. […] In-person, center-based PR is at this time considered the most effective and safest model of PR in the US. […] A small number of programs offer virtual or video-based PR. Virtual PR is a relatively new option in the US and is not usually paid for by Medicare for people with IPF and PF. […] If you are able to safely access an in-person program, you should discuss this option with your healthcare provider, but virtual programs may offer options for appropriate participants.

• #24 Pulmonary Fibrosis Treatment Options | Temple Health

  https://www.templehealth.org/services/conditions/pulmonary-fibrosis/treatment-options

  Depending on the cause and severity of your PF, you may be prescribed medication therapy to help relieve symptoms and slow the progression of your PF. […] Nintedanib (Ofev) and pirfenidone (Esbriet) are FDA-approved for patients with PF. These medications work to slow down the scarring (fibrosis) of the lungs caused by pulmonary fibrosis. […] Your pulmonologist may prescribe anti-inflammatories such as steroids to help decrease inflammation. […] Like steroids and anti-fibrotic agents, other medications may work to reduce inflammation by suppressing the immune system. […] Pulmonary rehabilitation is a standard treatment for people with chronic lung disease. […] Pulmonary rehab does not replace medical therapy. It’s used alongside medical treatments to help patients better manage their conditions and function at their best. […] Temple offers a comprehensive pulmonary rehabilitation program to support you in your efforts. […] Pulmonary fibrosis clinical trials are research studies designed to explore the effectiveness and safety of potential new treatments.

• #25 Pulmonary Fibrosis Causes, Symptoms, Stages & Life Expectancy

  https://www.medicinenet.com/pulmonary_fibrosis/article.htm

  What about pulmonary rehabilitation for pulmonary fibrosis? Pulmonary rehabilitation along with medical care helps improve the quality of life of people who have chronic breathing problems such as pulmonary fibrosis. It involves: Exercise conditioning, Nutrition counseling, Patient education on managing the disease, Breathing strategies, Techniques to help conserve energy, Counseling and/or support groups. […] Patients diagnosed with idiopathic pulmonary fibrosis who are current smokers are encouraged to quit. It is also recommended that patients with IPF receive vaccination against influenza and pneumococcal infection.

• #26 Pulmonary Fibrosis: Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/10959-pulmonary-fibrosis

  No, there isnt a cure for pulmonary fibrosis. Treatment focuses on easing symptoms, slowing down progression, and improving your quality of life. […] Treatments for pulmonary fibrosis could include: […] Antifibrotic medications. Nintedanib (OFEV) or pirfenidone (Esbriet) may slow down lung scarring and preserve lung function. […] Corticosteroids. These are sometimes used to reduce inflammation. […] Oxygen therapy. Your provider will prescribe extra oxygen if you dont have enough of it getting to your blood or tissues. Its delivered through a mask or tube in your nose. […] Pulmonary rehabilitation. Breathing exercises and physical therapy can make your lungs stronger and breathing easier. […] Lung transplant. Some people with fibrosis are eligible for a lung transplant. […] Clinical trials. If youre interested, your provider can help you join a research study of new medicines that can potentially help people. […] You may also need medications to treat underlying causes (like autoimmune disease) or conditions that make scarring worse (like GERD).

• #27

  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 your condition continues to get worse despite treatment, your specialist may recommend a lung transplant. […] A lung transplant can significantly improve life expectancy in people with IPF, although it’s a major procedure that puts a great strain on the body. […] 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.

• #28 Idiopathic Pulmonary Fibrosis | AAFP

  https://www.aafp.org/family-physician/patient-care/care-resources/respiratory-health/idiopathic-pulmonary-fibrosis.html

  Patients with IPF should be assessed for the need for oxygen therapy. This is best accomplished by a six-minute walk study. If the patients oxygen saturation drops below 88%, then an oxygen titration component should be performed. […] Lung transplantation is a potential option for patients with moderate to severe IPF, and it is the only treatment associated with prolonged survival.

• #29 Idiopathic pulmonary fibrosis: Current and future treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9060042/

  Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized by dry cough, fatigue, and progressive exertional dyspnea. […] The mainstay therapies are the oral antifibrotic drugs pirfenidone and nintedanib, which can improve quality of life, attenuate symptoms, and slow disease progression. […] Unilateral or bilateral lung transplantation is the only treatment for IPF shown to increase life expectancy. […] Clearly, there is an unmet need for accelerated research into IPF mechanisms so that progress can be made in therapeutics toward the goals of increasing life expectancy, alleviating symptoms, and improving wellbeing. […] Since there is no cure, treatment has remained focused on slowing progression of fibrosis, maintaining comfort and, in late stages, on palliative care.

• #30 Idiopathic pulmonary fibrosis: Current and future treatment

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9060042/

  Before transplant, antifibrotic treatment is generally maintained and strict adherence to medication regimen is encouraged. […] The first human clinical study on the pharmacokinetics and safety of PRM151, a recombinant human PTX2 protein, as a treatment for IPF, was a modest randomized, blinded, placebo-controlled trial. […] Pamrevlumab is a fully human recombinant monoclonal antibody that targets connective tissue growth factor (CTGF) and thus can possibly limit fibrotic progression. […] A phase 3 efficacy and safety study of PRM151 is in progress with estimated completion date of March 2023. […] There is an urgent need for more attention and in-depth research into development of targeted treatments that prolong life and improve quality of life for persons with IPF.

• #31 Pulmonary Fibrosis Treatment Shows Proof of Principle – Research Horizons

  https://scienceblog.cincinnatichildrens.org/pulmonary-fibrosis-treatment-shows-proof-of-principle/

  Pulmonary Fibrosis Treatment Shows Proof of Principle […] A pre-clinical study led by scientists at Cincinnati Children’s demonstrates that, in mice, the drug barasertib reverses the activation of fibroblasts that cause dangerous scar tissue to build up in the lungs of people with idiopathic pulmonary fibrosis (IPF). […] The discovery suggests that a powerful treatment for a fatal disease that currently has no cure other than lung transplantation may be within a few years of launching human clinical trials. […] “This study is the first to identify barasertib as an anti-fibrotic candidate,” Madala says. “That’s important because so far there are no treatments for IPF that appear to reverse the underlying process that causes the disease.” […] Those treated with barasertib before developing lung fibrosis were less likely to do so once scar formation was induced. Once fibrosis had started, introducing the drug significantly slowed further disease progress, primarily by causing faster cell death among the fibroblasts. The results of treatment included less scar tissue, improved lung elasticity, and overall better lung function.

• #32 Innovative Therapy Shows Promise in Reversing Lung Scarring in Idiopathic Pulmonary Fibrosis | The University of Arizona College of Medicine – Phoenix

  https://phoenixmed.arizona.edu/newsroom/news/innovative-therapy-shows-promise-reversing-lung-scarring-idiopathic-pulmonary

  „Whats exciting about FN-2012 is its ability to break up lung scarring, a function current therapies cannot achieve. Our studies show up to a 90% reduction in lung fibrosis in preclinical models. Thats unprecedented,” he explained. […] „When treated with FN-2012, we see a progressive loss of collagen and fibronectin, which are the major components of fibrotic scarring. Unlike other drugs, which still show an increase in scarring over time, FN-2012 is actively reducing it,” Dr. Marlowe added. […] „If this therapy works, it could be applied to other lung fibrosis conditions and even fibrotic diseases in other organs. It could be a game-changer.” […] „The results were remarkable, showing significant reductions in fibrosis across multiple models,” Dr. Knox noted. […] „Our main goal is to prepare for an Investigational New Drug (IND) application, which is required before we can begin human clinical trials. We are conducting rigorous safety and toxicity studies to ensure FN-2012 meets all FDA requirements.” Dr. Marlowe said.

• #33 Study suggests a new way to treat incurable lung disease – VUMC News

  https://news.vumc.org/2025/01/08/study-suggests-a-new-way-to-treat-incurable-lung-disease/

  Researchers at Vanderbilt University Medical Center and the University of Michigan Medical School have identified, in lab studies, a potential new treatment for idiopathic pulmonary fibrosis (IPF), a progressive, incurable lung disease that is on the rise in the United States. […] The most exciting part of this work, said McCall, is that it demonstrates a new therapeutic approach with the potential to be disease-modifying for IPF. […] Administration of small-molecule HIF2 inhibitor PT-2385 also reduced fibrosis and the appearance of abnormal epithelial cells and promoted alveolar repair. […] Together, these studies showed that HIF2 activation drives the emergence of aberrant epithelial populations after repetitive injury and that targeted HIF2-inhibition may represent an effective therapeutic strategy to promote functional alveolar repair in IPF and other interstitial lung diseases, the researchers concluded.

• #34 New research offers hope for IPF treatment | Royal Brompton & Harefield hospitals

  https://www.rbht.nhs.uk/new-research-offers-hope-for-ipf-treatment

  Researchers have discovered that blocking a protein called interleukin-11 (IL-11) can reverse scarring in Idiopathic Pulmonary Fibrosis (IPF). […] Currently there are two drugs, pirfenidone and nintedanib, which can help slow the rate at which the disease progresses. There is currently no treatment that can stop or reverse scarring that has already formed in the lung. […] Using mice models the researchers administered drugs, known as therapeutic antibodies, which attach to IL-11, effectively blocking its activity. The researchers found that not only did the drugs reduce damage to the lungs but it also reversed scarring that had already formed. […] Although we have treatments to slow disease progression, we desperately need new therapies to genuinely transform outcomes for people with IPF. This exciting research highlights the importance of IL-11 in driving the development of fibrosis and gives hope for a new treatment approach to halt and maybe even reverse the devastating lung scarring of IPF.

• #35 Newly Designed Molecule Is Promising Potential Therapy for Deadly Lung Condition < Yale School of Medicine

  https://medicine.yale.edu/news-article/newly-designed-molecule-is-promising-potential-therapy-for-deadly-lung-condition/

  Idiopathic pulmonary fibrosis (IPF) is a deadly condition. The only available therapies can slow disease progression, but they are not a cure and often cause intolerable side effects. […] A team led by Kaminski used a newly designed molecule, called MRG-229, with potential therapeutic implications for IPF. […] In 2014, Kaminski’s team published its work on a first-generation miR-29 mimic, Remlarsen/MRG-201, which showed that high doses of the miR-29 mimic could reduce fibrosis. […] Using multiple models, the team then studied their latest microRNA mimic’s ability to reduce fibrosis. […] With this model, we started to see MRG-229 as a viable option for trials in humans, says Kaminski. […] We proved in multiple models—in vivo, ex vivo, and in vitro—that through using a microRNA mimic we could reverse fibrosis, he says.

• #36 Newly Designed Molecule Is Promising Potential Therapy for Deadly Lung Condition < Yale School of Medicine

  https://medicine.yale.edu/news-article/newly-designed-molecule-is-promising-potential-therapy-for-deadly-lung-condition/

  After finding promising results across several models, the team next ran several toxicological studies in rats and non-human primates to assess the molecule’s safety as a potential therapeutic. […] Kaminski feels that his team’s work is powerful evidence in support of MRG-229 as a therapeutic. […] Because MRG-229 is designed with a targeting receptor to limit its impact outside the lung and was shown to be safe and effective in animals, the team is hopeful that the molecule will be tolerable in human patients. […] MRG-229 is approaching the end of the preclinical stage of drug development. […] We hope that the publication of our work will reawaken interest in the potential clinical development of miR-29 as a therapy in the lungs and possibly other organs he says. […] I really hope it happens.

• #37 Immunotherapy shows potential in treating lung fibrosis | News Center

  https://med.stanford.edu/news/all-news/2020/06/immunotherapy-shows-potential-in-treating-lung-fibrosis-.html

  New research suggests that lung fibrosis develops when scar tissue cells escape immune surveillance, suggesting potential therapy. […] A therapy may lie in reactivating that immune function. […] „This study presents hope for a new treatment option that could improve the condition of patients with lung fibrosis,” Wernig said. Currently, the only remedy for lung fibrosis is a lung transplant, she notes. […] When the researchers blocked signaling by IL-6 and CD47 simultaneously, the treatment dramatically improved lung tissue in mice by increasing the removal of fibrotic cells. „In mice, with the combined blockade of inflammation and the 'don’t eat me’ signal, we were able to eliminate established, end-stage fibrosis,” Wernig said.

• #38 Zinc Found to Play an Important Role in Lung Fibrosis

  https://www.cedars-sinai.org/newsroom/zinc-found-to-play-an-important-role-in-lung-fibrosis/

  A new study shows that zinc, a common mineral, may reverse lung damage and improve survival for patients with idiopathic pulmonary fibrosis (IPF). […] Their findings, published in The Journal of Clinical Investigation, have the potential to change the landscape of treatment for patients with this disease, which most often affects those over age 50. […] We identified a root cause of IPF-related lung damage and a potential therapeutic target that might restore the lungs ability to heal themselves. […] Restoring this ability via the molecular pathway the investigators traced in their experiments could lead to therapies that reverse IPF-related lung damage. […] Additional research is needed to help determine why the loss of ZIP8 occurs in the lung cells, and whether zinc supplements, alone or in combination with NAD+ and Sirtuin1 activators to help activate lung repair response, will reverse lung damage in human patients with this condition. […] The goal is to develop a clinical trial to determine if targeting the ZIP8/NAD+/Sirtuin1 pathway can improve lung function in IPF.

• #39 How Is Pulmonary Fibrosis Treated? | American Lung Association

  https://www.lung.org/lung-health-diseases/lung-disease-lookup/pulmonary-fibrosis/patients/how-is-pulmonary-fibrosis-treated

  Lung transplant evaluation should be recommended by your physician early after diagnosis. […] Clinical trials are being conducted to better understand how pulmonary fibrosis develops and to advance the treatments available. Clinical trials may give you access to new types of treatment being studied. […] Treatment is only one part of living well with PF. Nutrition, exercise, stress management and protecting your lungs all have an impact on your disease.

• #40 Pulmonary Fibrosis and Interstitial Lung Disease – Pulmonary, Allergy and Critical Care Medicine

  https://www.uab.edu/medicine/pulmonary/about/programs/the-program-for-pulmonary-fibrosis-and-interstitial-lung-disease

  The Program for Pulmonary Fibrosis and Interstitial Lung Disease’s mission is to provide state of the art evaluation and treatment for patients with interstitial lung diseases (ILD). […] To lead efforts in scientific discovery that informs the development of new and effective therapies for interstitial lung diseases, particularly idiopathic pulmonary fibrosis (IPF) […] This program exists to better understand such illnesses, train physicians to more completely treat such illnesses, and to drive the creation of superior treatment options. […] Our entire team at the ILD clinic is dedicated to improving the lives of our patients with ILD through patient care, education and research. […] The UAB ILD Center offers clinical trial enrollment for patients with idiopathic pulmonary fibrosis and other forms of diffuse parenchymal lung disease as part of a multidisciplinary and integrated approach to treatment.

• #41 Pulmonary fibrosis treatment options – Breathing Matters – UCL Respiratory

  https://www.breathingmatters.co.uk/pulmonary-fibrosis-treatment-options-and-outcomes/

  Managing symptoms is crucial for sustaining the quality of life in patients with lung fibrosis. The goal for treating idiopathic pulmonary fibrosis (IPF) is to alleviate symptoms and slow the advancement of the disease. […] Treatments for pulmonary fibrosis often involve oxygen therapy, which is vital for elevating the bloods oxygen concentration and bolstering routine activities. […] The objective of palliative care is to improve the quality of life for patients by easing the symptoms and stress that come with severe diseases, including pulmonary fibrosis. […] Managing lung fibrosis involves a multifaceted approach that includes lifestyle changes, medication, symptom management and, in some cases, surgical interventions like lung transplant. Staying informed and proactive in treatment can significantly improve quality of life.

• #42 Palliative Care and Pulmonary Fibrosis | Get Palliative Care

  https://getpalliativecare.org/whatis/disease-types/pulmonary-fibrosis-and-palliative-care/

  Palliative care can make a big difference. Some palliative care treatments for pulmonary fibrosis include medications that relieve symptoms such as shortness of breath. […] Oxygen therapy can make breathing and sleep easier. […] Palliative care specialists are experts in treating the symptoms of pulmonary fibrosis and the side effects of treatment. […] If you or a loved one is facing pulmonary fibrosis, ask your doctor for a referral to palliative care the earlier the better. […] Although living with pulmonary fibrosis is a difficult journey, palliative care can ease your burden and help you achieve the best possible quality of life.

• #43 Idiopathic Pulmonary Fibrosis (IPF) Treatment & Management: Approach Considerations, Medical Care, Surgical Care

  https://emedicine.medscape.com/article/301226-treatment

  Improving quality of life is an important goal in disease management. […] Lung transplantation for idiopathic pulmonary fibrosis has been shown to confer a survival benefit over medical therapy. […] The clinical course of patients with idiopathic pulmonary fibrosis (IPF) is generally marked by a decline in pulmonary function over time. […] Corticosteroids are recommended for the treatment of the majority of patients with AE-IPF by the most recent international idiopathic pulmonary fibrosis treatment guidelines in 2022. […] Early palliative care referral is recommended as an adjunct to disease-focused care in idiopathic pulmonary fibrosis (IPF).

• #44 Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat | Respiratory Research | Full Text

  https://respiratory-research.biomedcentral.com/articles/10.1186/s12931-019-1161-4

  Management of the side-effects that may occur when they take antifibrotic therapy is important to helping patients stay on treatment. […] Education is key to patients understanding the role of antifibrotic therapies in reducing disease progression, so that they can make an informed assessment of the benefits of therapy in the context of side effects that may occur. […] In addition to anti-fibrotic therapies, patients with IPF benefit from a holistic approach to care that may include pulmonary rehabilitation, symptom management, education and support, vaccinations, management of comorbidities, supplemental oxygen for those with hypoxemia, and palliative care tailored to the needs of the patient and their caregivers. […] Prompt treatment of IPF is critical to preserving individuals lung function, reducing the risk of acute exacerbations and improving outcomes.

• #45 A Review of the Treatment and Management of Idiopathic Pulmonary Fibrosis

  https://www.uspharmacist.com/article/a-review-of-the-treatment-and-management-of-idiopathic-pulmonary-fibrosis

  Supportive care is an integral component of IPF treatment and management and can include supplemental oxygen, pulmonary rehabilitation, patient education about IPF and its management and the importance of obtaining recommended vaccines, including the seasonal influenza and pneumococcal vaccinations, and palliative care throughout all stages of the disease. […] Treatment of IPF is multifaceted, and pharmacists can be influential in recommending evidence-based pharmacologic agents when medication is warranted in IPF patients. […] During counseling, it is crucial to advise patients on what to expect from prescribed therapies, any precautions, and any potential adverse effects they may experience and how to manage them. […] It is important that pharmacists counsel patients on the pharmacologic action of the selected agent, dosing, and administration and review the most common and serious adverse drug reactions.

• #46 A Review of the Treatment and Management of Idiopathic Pulmonary Fibrosis

  https://www.uspharmacist.com/article/a-review-of-the-treatment-and-management-of-idiopathic-pulmonary-fibrosis

  Disease-management programs are especially significant when dealing with a progressive disease like IPF, since patients may experience an overwhelming lack of psychosocial support, educational resources, and information regarding treatment options, patient drug-assistance programs, supplemental oxygen, pulmonary rehabilitation, and transplantation. […] A collaborative effort between pharmacists, pulmonary professionals, and patients/caregivers, coupled with patient education and emphasizing the vital nature of patient adherence, is fundamental for efficaciously managing IPF, improving patient quality of life, and delaying the progression of this relentless pulmonary condition.

• #47 Pulmonary Fibrosis Program | Temple Health

  https://www.templehealth.org/services/lung/patient-care/programs/pulmonary-fibrosis

  Specjaliści w Temple Lung Center leczą setki pacjentów każdego roku w ramach naszego Programu Pulmonary Fibrosis. […] Chociaż nie ma jeszcze lekarstwa na włóknienie płuc, Temple oferuje obiecujące nowe terapie w połączeniu z długoterminowym wsparciem, aby pomóc naszym pacjentom żyć dłużej i lepiej oddychać, zapewniając jak najlepszą jakość życia. […] Program Pulmonary Fibrosis Temple oferuje dostęp do najnowszych leków, które pomagają spowolnić postęp bliznowacenia płuc, w tym leki przeciwfibrotyczne, takie jak nintedanib i pirfenidon. […] Nasz zespół Pulmonary Fibrosis spotyka się co miesiąc, aby eksperci z różnych specjalności, w tym reumatologii, pulmonologii, patologii i radiologii, mogli omawiać i współpracować w zakresie diagnozy i planów leczenia naszych pacjentów. […] Program Pulmonary Fibrosis Temple jest zobowiązany do pomocy pacjentom z tą chorobą układu oddechowego. […] Jeśli doświadczasz objawów włóknienia płuc, umów się na wizytę lub zadzwoń pod numer 800-TEMPLE-MED (800-836-7536) już dziś.

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