Materiały źródłowe

• #1 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. […] Since there is no cure, treatment has remained focused on slowing progression of fibrosis, maintaining comfort and, in late stages, on palliative care. […] Two antifibrotic therapies have been approved for the treatment of IPF: pirfenidone and nintedanib. […] Either nintedanib or pirfenidone are good choices as the primary treatment for IPF at the present time.

• #2 Pulmonary Fibrosis: Causes, Symptoms & Treatment

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

  Healthcare providers can’t easily predict how pulmonary fibrosis will progress. Your symptoms may get worse very slowly, over years. In some cases, the disease may lead to severe symptoms quickly (over months). […] The life expectancy of someone with the most common form, idiopathic pulmonary fibrosis, is three to five years. But life expectancies for people with PF have been getting longer in recent years. […] It’s important to remember that every case of PF is different, and no one can tell you what your future holds. Treatment can sometimes slow down the progression of pulmonary fibrosis and improve your quality of life.

• #3 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. […] 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. […] 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.

• #4

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

  Several treatments can help reduce the rate at which IPF gets worse, but there’s currently no treatment that can stop or reverse the scarring of the lungs. […] There’s currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression. […] Treatments include: self care measures, such as stopping smoking, eating healthily and exercising regularly; medicines to reduce the rate at which scarring worsens, such as pirfenidone and nintedanib; breathing oxygen through a mask you can do this while you’re at home or while you’re out and about; exercises and advice to help you breathe more easily (pulmonary rehabilitation); a lung transplant this is suitable in a few cases, although donor lungs are rare.

• #5

  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.

• #6 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).

• #7 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. […] Since there is no cure, treatment has remained focused on slowing progression of fibrosis, maintaining comfort and, in late stages, on palliative care. […] Two antifibrotic therapies have been approved for the treatment of IPF: pirfenidone and nintedanib. […] Either nintedanib or pirfenidone are good choices as the primary treatment for IPF at the present time.

• #8 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

  There are two approved therapies used to slow the progression of the disease. […] In 2014, the FDA approved the antifibrotics nintedanib and pirfenidone in a noteworthy breakthrough in the treatment of IPF. […] The efficacy data of pirfenidone and nintedanib demonstrate the ability of both of these agents to slow down the decline of lung function in IPF patients. […] Nintedanib is a multitargeted tyrosine kinase inhibitor that is indicated for the treatment of IPF. […] Pirfenidone is classified as a pyridone indicated for the treatment of IPF. […] 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.

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

  https://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). […] Nintedanib is a triple tyrosine kinase receptor inhibitor that has anti-platelet-derived growth factor activity (PDGF), anti-fibroblast growth factor (FGF) activity, and anti-vascular endothelial growth factor (VEGF) activity, all associated with the pathogenic mechanisms of fibrosis. The benefit of nintedanib in IPF is supported by 3 double-blind, placebo-controlled randomized trials.

• #10 Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age – Shaw – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/16911/html

  Pirfenidone is an immunosuppressive agent designed to specifically target the underlying disease mechanism in IPF. Although its mechanism of action is not fully understood, it is likely that pirfenidone prevents fibroblast proliferation and therefore prevents the release of pro-fibrotic and pro-inflammatory mediators. […] Two randomised control trials (RCT) underpin the evidence base for pirfenidone in IPF. The CAPACITY-1 and CAPACITY-2 trials compared decline in FVC in patients taking pirfenidone or placebo in mild to moderate IPF. […] The second ASCEND study was requested by the United States Food and Drug Administration (US FDA) due to the inconsistent findings in the CAPACITY trials in meeting the primary endpoint. This RCT corroborated the initial findings, again showing a reduction in FVC decline in patients using pirfenidone compared to placebo.

• #11 Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age – Shaw – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/16911/html

  Pirfenidone received approval from the US FDA in 2014, having previously received approval from the European Medicines Agency (EMA) in 2010. The National Institute of Health and Care Excellence (NICE) approved use of pirfenidone in the UK in April 2013. […] Nintedanib is a tyrosine kinase inhibitor and causes inhibition of intracellular growth factor signalling. This pathway is thought to be involved in the development of fibrosis and its inhibition leads to a reduction in pro-fibrotic mediators. […] Nintedanib received US FDA approval on the same day as pirfenidone in October 2014 and EMA approval in November 2014. NICE approved use of nintedanib in the UK in January 2016. […] Both pirfenidone and nintedanib have similar effects on lung function with pirfenidone showing a mortality benefit in pooled data. The decision as to which treatment to start is based on joint decision making between patient and physician.

• #12 Treating and managing IPF | Asthma + Lung UK

  https://www.asthmaandlung.org.uk/conditions/idiopathic-pulmonary-fibrosis-ipf/treating-managing-ipf

  Treatment for IPF aims to slow the rate of scarring in the lung and treat the symptoms. […] There are currently two drugs that can be prescribed to slow down the rate of scar tissue developing in the lungs: Pirfenidone and Nintedanib. […] Current studies suggest both drugs are equally effective in slowing down the rate of scarring in the lungs. […] This treatment comes in the form of capsules or tablets taken with meals. Clinical trials found that the drug slowed down the loss of lung function in most people with IPF, slowed the rate at which their symptoms got worse, and improved life expectancy. […] Nintedanib has also been shown in trials to slow the rate at which lungs become scarred in IPF. […] The National Institute for Health and Care Excellence (NICE) has only recommended the use of pirfenidone and nintedanib for people whose lung function is within a certain range.

• #13 Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age – Shaw – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/16911/html

  Pirfenidone received approval from the US FDA in 2014, having previously received approval from the European Medicines Agency (EMA) in 2010. The National Institute of Health and Care Excellence (NICE) approved use of pirfenidone in the UK in April 2013. […] Nintedanib is a tyrosine kinase inhibitor and causes inhibition of intracellular growth factor signalling. This pathway is thought to be involved in the development of fibrosis and its inhibition leads to a reduction in pro-fibrotic mediators. […] Nintedanib received US FDA approval on the same day as pirfenidone in October 2014 and EMA approval in November 2014. NICE approved use of nintedanib in the UK in January 2016. […] Both pirfenidone and nintedanib have similar effects on lung function with pirfenidone showing a mortality benefit in pooled data. The decision as to which treatment to start is based on joint decision making between patient and physician.

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

  https://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). […] Nintedanib is a triple tyrosine kinase receptor inhibitor that has anti-platelet-derived growth factor activity (PDGF), anti-fibroblast growth factor (FGF) activity, and anti-vascular endothelial growth factor (VEGF) activity, all associated with the pathogenic mechanisms of fibrosis. The benefit of nintedanib in IPF is supported by 3 double-blind, placebo-controlled randomized trials.

• #15 Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age – Shaw – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/16911/html

  Pirfenidone received approval from the US FDA in 2014, having previously received approval from the European Medicines Agency (EMA) in 2010. The National Institute of Health and Care Excellence (NICE) approved use of pirfenidone in the UK in April 2013. […] Nintedanib is a tyrosine kinase inhibitor and causes inhibition of intracellular growth factor signalling. This pathway is thought to be involved in the development of fibrosis and its inhibition leads to a reduction in pro-fibrotic mediators. […] Nintedanib received US FDA approval on the same day as pirfenidone in October 2014 and EMA approval in November 2014. NICE approved use of nintedanib in the UK in January 2016. […] Both pirfenidone and nintedanib have similar effects on lung function with pirfenidone showing a mortality benefit in pooled data. The decision as to which treatment to start is based on joint decision making between patient and physician.

• #16

  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.

• #17 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

  There are two approved therapies used to slow the progression of the disease. […] In 2014, the FDA approved the antifibrotics nintedanib and pirfenidone in a noteworthy breakthrough in the treatment of IPF. […] The efficacy data of pirfenidone and nintedanib demonstrate the ability of both of these agents to slow down the decline of lung function in IPF patients. […] Nintedanib is a multitargeted tyrosine kinase inhibitor that is indicated for the treatment of IPF. […] Pirfenidone is classified as a pyridone indicated for the treatment of IPF. […] 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.

• #18 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

  In the US, Europe and many other countries, two drugs are approved for the treatment of IPF: nintedanib and pirfenidone. […] 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.

• #19 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

  In the US, Europe and many other countries, two drugs are approved for the treatment of IPF: nintedanib and pirfenidone. […] 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.

• #20

  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. […] 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.

• #21 Antifibrotic treatment improves clinical outcomes in patients with idiopathic pulmonary fibrosis: a propensity score matching analysis | Scientific Reports

  https://www.nature.com/articles/s41598-020-72607-1

  Antifibrotic treatment significantly reduced the risks of mortality [hazard ratio (HR), 0.59; 95% confidence interval (CI), 0.480.72; p0.001], all-cause hospitalisation (HR 0.71), respiratory-related hospitalisation (HR 0.67), acute exacerbation (HR 0.69), and mortality after acute exacerbation (HR 0.60). […] Our results suggest that antifibrotic treatment may reduce the risks of all-cause mortality, hospitalisation, acute exacerbation, and mortality after acute exacerbation in patients with IPF. […] In the univariate Cox analysis, antifibrotic treatment was associated with a significantly reduced risks of mortality [hazard ratio (HR), 0.59; 95% CI, 0.480.72; p0.001], all-cause hospitalisation (HR 0.71; 95% CI 0.570.88; p=0.002), respiratory-related hospitalisation (HR 0.67; 95% CI 0.520.86; p=0.002), AE (HR 0.69; 95% CI 0.500.96; p=0.026), and mortality following AE (HR 0.60; 95% CI 0.420.85; p=0.004) in IPF patients.

• #22 Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age – Shaw – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/16911/html

  Patients expectations of a new treatment need to be addressed by advising them that the antifibrotic treatments have not been shown to improve breathlessness. […] The choice of antifibrotic is a joint decision between patient and physician after discussing the relative potential side effect profiles and contraindications of each treatment. […] Both pirfenidone and nintedanib have side effect profiles that require careful education and management to maintain adherence and maximise efficacy. […] The majority of antifibrotic side effects are gastro-intestinal in nature. […] Ongoing side effects may require supervised dose reduction and re-titration once the symptoms have subsided. […] Both the CAPACITY and ASCEND trials found similar side effect profiles with pirfenidone. […] The INPULSIS trials similarly showed that most adverse events experienced with nintedanib are mild to moderate in nature.

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

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

  When a diagnosis of IPF has been established, antifibrotic medication should begin as soon as possible in order to delay functional decline that would occur without treatment. In asymptomatic patients with minimal functional or radiological impact, a wait-and-see approach can be taken, after the risks and benefits of such a strategy have been weighed up. […] The main contraindications for the use of pirfenidone are drug hypersensitivity, concomitant use of fluvoxamine, and severe liver or kidney disease. […] The most common adverse effect of nintedanib, occurring in up to 60% of cases, is diarrhea, generally mild-to-moderate in intensity. In only about 4%-5% of patients is it sufficiently severe or recurrent to require definitive withdrawal of the drug. Other less common effects are nausea and vomiting, anorexia, weight loss, and changes in hepatic function.

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

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

  When a diagnosis of IPF has been established, antifibrotic medication should begin as soon as possible in order to delay functional decline that would occur without treatment. In asymptomatic patients with minimal functional or radiological impact, a wait-and-see approach can be taken, after the risks and benefits of such a strategy have been weighed up. […] The main contraindications for the use of pirfenidone are drug hypersensitivity, concomitant use of fluvoxamine, and severe liver or kidney disease. […] The most common adverse effect of nintedanib, occurring in up to 60% of cases, is diarrhea, generally mild-to-moderate in intensity. In only about 4%-5% of patients is it sufficiently severe or recurrent to require definitive withdrawal of the drug. Other less common effects are nausea and vomiting, anorexia, weight loss, and changes in hepatic function.

• #25 Idiopathic pulmonary fibrosis: a holistic approach to disease management in the antifibrotic age – Shaw – Journal of Thoracic Disease

  https://jtd.amegroups.org/article/view/16911/html

  Both antifibrotics can cause elevation of liver enzymes and so liver function tests (LFTs) should be measured at baseline and throughout treatment. […] The ILD specialist nurses provide an important link between the MDT and the general practitioner. Specialist nurses and support strategies such as IPF care provide a key resource and support for patients. […] Over the past decade there have been significant changes to the management of IPF following the advent of two antifibrotic therapies. Pirfenidone and nintedanib offer potential prognostic benefit in an otherwise deadly disease. Both drugs have significant but manageable side effect profiles and specialists managing patients with IPF need to take this into consideration when selecting the right drug for the right patient.

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

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

  In October 2014, the US Food and Drug Administration (FDA) approved nintedanib (Ofev) for treatment of idiopathic pulmonary fibrosis. […] A phase 3 multicenter, double-blind, placebo-controlled, randomized clinical trial in Japan examined the use of pirfenidone. […] The FDA approved pirfenidone (Esbriet) for the treatment of idiopathic pulmonary fibrosis in October 2014. […] To date there is no compelling evidence to support the dual use of pirfenidone and nintedanib for the management of idiopathic pulmonary fibrosis. […] Patients with hypoxemia (PaO2 55 mmHg or oxygen saturation as measured using pulse oximetry [SpO2] 88%) at rest or with exercise should be prescribed oxygen therapy to maintain a saturation of at least 90% at rest, with sleep, and with exertion. […] Improving quality of life is an important goal in disease management.

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

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

  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.

• #28 Idiopathic Pulmonary Fibrosis | AAFP

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

  The goals of treatment are to slow progression of the disease, reduce symptoms, and improve the quality of life. IPF is currently treated with a combination of antifibrotic drugs and pulmonary rehabilitation. The need for oxygen therapy should be assessed, and lung transplantation is an option for moderate to severe disease in select patients. Clinical trials and registries may be available for patient involvement in your area. […] Until 2014, no approved drugs were available for the treatment of IPF. Now, two first-in-class antifibrotic drugs are approved by the Food and Drug Administration (FDA). Studies have shown both drugs slow disease progression in patients with IPF, as measured by the decline in FVC. […] A systematic review of nine studies demonstrated that pulmonary rehabilitation is beneficial for people with interstitial lung disease, including IPF. According to the findings, pulmonary rehabilitation was safe and was associated with short-term improvements in functional exercise capacity, dyspnea, and quality of life.

• #29 Pulmonary Fibrosis Treatment

  https://uvahealth.com/services/pulmonary/pulmonary-fibrosis

  Pulmonary rehabilitation helps you manage shortness of breath. Rehabilitation is tailored to your needs but may include: Exercises, Nutrition, Education, Breathing techniques and coping strategies, Support from others. […] If other treatments fail, you may need a lung transplant. A transplant can improve both the quality and length of your life. […] At UVA Health, you’ll find the comprehensive pulmonary fibrosis treatment needed to help you breathe easier.

• #30 Pulmonary Fibrosis Treatment Options | Temple Health

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

  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. […] Stopping smoking immediately is very important for all patients with idiopathic pulmonary fibrosis. […] 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. […] Every day, researchers with Temples Interstitial Lung Disease Program strive to make new discoveries in the areas of pulmonary fibrosis treatment, including devices, behavioral therapies, testing and diagnostic methods, and medications.

• #31

  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.

• #32 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. […] Since there is no cure, treatment has remained focused on slowing progression of fibrosis, maintaining comfort and, in late stages, on palliative care. […] Two antifibrotic therapies have been approved for the treatment of IPF: pirfenidone and nintedanib. […] Either nintedanib or pirfenidone are good choices as the primary treatment for IPF at the present time.

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

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

  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.

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

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

  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).

• #35 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.

• #36 Idiopathic Pulmonary Fibrosis: Overview – Life Extension

  https://www.lifeextension.com/protocols/respiratory/idiopathic-pulmonary-fibrosis?srsltid=AfmBOoqYJpTBxhyCNW56rNJdWzQG8N9OnJSIvFUjXjgRnNlkGwdLp2_i

  A lung transplant may increase survival time and is an important option in appropriate patients with moderate-to-advanced IPF. […] IPF patients frequently suffer from other chronic conditions. Emphysema, lung cancer, pulmonary hypertension, sleep apnea, gastroesophageal reflux, and coronary artery disease are among the most common co-occurring conditions. […] Proton pump inhibitors (PPIs), usually used to treat gastroesophageal reflux, are of interest in IPF research as well. Some studies have found that they have anti-fibrotic properties, and that IPF patients even without GERD symptoms often have elevated esophageal acid levels. […] Clinical trials can give patients access to emerging and experimental treatments that are not yet approved or widely available, such as those described in the „Novel and Emerging Interventions” section of this protocol.

• #37 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

  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). Corticosteroids can have harmful side effects, especially when used long term. You will need to be carefully monitored if you are on long-term steroids. 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. There are different types of anti-acid medications (e.g., Proton pump inhibitors including Prilosec OTC and Nexium as well as H2-Blockers including Zantac and Pepcid) that are prescribed to help block the formation of or minimize the amount of acid in the stomach. By doing so, it helps to reduce the amount of acid from the stomach that enters the lungs.

• #38 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

  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. There are ongoing clinical trials for PF drugs, including ones to help relieve cough. Visit clinicaltrials.gov to see if any are available near you or ask your doctor.

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

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

  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).

• #40

  https://www.pulmonaryfibrosis.org/patients-caregivers/medical-and-support-resources/clinical-trials-education-center

  Clinical trials for pulmonary fibrosis treatment are research studies that explore whether a medical strategy, treatment, or device is safe and effective for humans. These studies also may show which medical approaches work best for certain illnesses or groups of people. Clinical trials for pulmonary fibrosis treatment produce the best data available for health care decision making. […] With studies currently in various stages of development, there are a variety of clinical trials that are actively seeking the participation of patients. Some of the therapeutic approaches currently being studied include: Anti-fibrotic therapies, which may slow or inhibit the production of scar tissue (fibrosis). […] What types of research are being performed in clinical trials for pulmonary fibrosis treatment? Basic science studies are done to increase the fundamental understanding of the disease process on a molecular and cellular level. In the long term, this type of research can provide the scientific basis for the development of effective treatments. […] Clinical research studies are performed to determine the safety and efficacy of medications, treatment regiments, and diagnostic procedures. The different phases of clinical trials for the development of new therapies are described above.

• #41 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. […] 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. […] 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.

• #42 Advancing Care for Idiopathic Pulmonary Fibrosis (IPF): Evaluating Current Treatments and Emerging Therapies for Improved Patient Outcomes

  https://www.ajmc.com/view/advancing-care-for-idiopathic-pulmonary-fibrosis-ipf-evaluating-current-treatments-and-emerging-therapies-for-improved-patient-outcomes

  High rates of AE-IPF, mortality, and adverse effect-related treatment discontinuation highlight the critical need for new antifibrotic therapies that improve disease prognosis and treatment tolerability, enabling better long-term adherence and improved patient outcomes in IPF management. […] PDE4 inhibitors are already being used for COPD to reduce exacerbations; however, there are none yet approved for IPF. […] Inhibiting PDE4 results in elevated levels of cAMP, thereby producing its antifibrotic effects. […] Preferential inhibition of PDE4B is being investigated for the treatment of IPF as it may retain its anti-inflammatory and antifibrotic properties while avoiding certain use-limiting adverse effects. […] Recent clinical trial data offer hope, showcasing emerging therapies that may slow disease progression and improve patient outcomes.

• #43 Treatment of idiopathic pulmonary fibrosis – UpToDate

  https://www.uptodate.com/contents/treatment-of-idiopathic-pulmonary-fibrosis

  • Which patients should be treated? […] • When should therapy be started? […] • What is the best therapy? […] […] […] MEDICAL THERAPIES […] Our approach […] Nintedanib […] – Dose and administration […] – Efficacy […] – Role in more advanced disease […] Pirfenidone […] – Dose and administration […] – Efficacy […] – Role in more advanced disease […] Clinical trials […] […] […] THERAPIES WITHOUT CLEAR BENEFIT […] Empiric treatment for asymptomatic gastroesophageal reflux […] Anticoagulation […] Maintenance antibiotic therapy […] Azathioprine, prednisone, and (N) acetylcysteine […] (N) acetylcysteine […] Other immunosuppressants […] Endothelin receptor antagonists […] Phosphodiesterase-5 inhibitors […] Collagen-modifying agents […] […] […] FUTURE DIRECTIONS […] Combination nintedanib plus pirfenidone […] Phosphodiesterase 4B inhibitor (BI 1015550) […] Lysophosphatidic acid receptor 1 antagonists (admilparant) […] Other agents

• #44 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.

• #45 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

  Researchers develop FN-2012, a novel treatment that could transform the management of IPF and other fibrotic diseases. […] „IPF is a rapidly progressive and highly fatal disease. There are only two FDA-approved drugs available, but they provide only modest benefits and come with severe side effects that often lead many patients to discontinue treatment. More effective and better-tolerated therapies are urgently needed,” Dr. Marlowe shared. […] „Current treatments don’t reverse fibrosis; they only slow its progression. Patients often struggle with taking these drugs because they dont feel any better, and the side effects can be overwhelming. We need a therapy that can not only halt but potentially reverse fibrosis.” […] „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.

• #46 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.

• #47 Treatment of idiopathic pulmonary fibrosis – UpToDate

  https://www.uptodate.com/contents/treatment-of-idiopathic-pulmonary-fibrosis

  • Which patients should be treated? […] • When should therapy be started? […] • What is the best therapy? […] […] […] MEDICAL THERAPIES […] Our approach […] Nintedanib […] – Dose and administration […] – Efficacy […] – Role in more advanced disease […] Pirfenidone […] – Dose and administration […] – Efficacy […] – Role in more advanced disease […] Clinical trials […] […] […] THERAPIES WITHOUT CLEAR BENEFIT […] Empiric treatment for asymptomatic gastroesophageal reflux […] Anticoagulation […] Maintenance antibiotic therapy […] Azathioprine, prednisone, and (N) acetylcysteine […] (N) acetylcysteine […] Other immunosuppressants […] Endothelin receptor antagonists […] Phosphodiesterase-5 inhibitors […] Collagen-modifying agents […] […] […] FUTURE DIRECTIONS […] Combination nintedanib plus pirfenidone […] Phosphodiesterase 4B inhibitor (BI 1015550) […] Lysophosphatidic acid receptor 1 antagonists (admilparant) […] Other agents

• #48 Idiopathic pulmonary fibrosis: new approaches to diagnosis and treatment | Respiratory Medicine Today

  https://respiratory.medicinetoday.com.au/rmt/2020/may/feature-article/idiopathic-pulmonary-fibrosis-new-approaches-diagnosis-and-treatment

  The comprehensive management of IPF involves disease-specific therapies and management of comorbidities, disease-related symptoms, pulmonary rehabilitation as well as palliative care and/or lung transplantation. […] Early referral is critical for both the diagnosis and management of IPF. […] Until recently, there were no effective treatments available for IPF. […] In 2014, two landmark trials of the antifibrotic therapies nintedanib and pirfenidone, respectively, were published. […] PBS criteria in Australia for use of antifibrotics include MDT diagnosis of IPF, HRCT consistent with UIP pattern within 12 months, FVC greater than 50%, FEV1/FVC more than 0.7, DLCO more than 30% and ILD not a result of another known cause. […] Lung transplant remains the only definitive cure for IPF. […] Palliative care is a key part of IPF management and should be addressed at all stages of the disease.

• #49 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.

• #50 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

  In the US, Europe and many other countries, two drugs are approved for the treatment of IPF: nintedanib and pirfenidone. […] 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.

• #51

  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. […] 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.

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

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

  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).

• #53 Idiopathic pulmonary fibrosis: new approaches to diagnosis and treatment | Respiratory Medicine Today

  https://respiratory.medicinetoday.com.au/rmt/2020/may/feature-article/idiopathic-pulmonary-fibrosis-new-approaches-diagnosis-and-treatment

  The comprehensive management of IPF involves disease-specific therapies and management of comorbidities, disease-related symptoms, pulmonary rehabilitation as well as palliative care and/or lung transplantation. […] Early referral is critical for both the diagnosis and management of IPF. […] Until recently, there were no effective treatments available for IPF. […] In 2014, two landmark trials of the antifibrotic therapies nintedanib and pirfenidone, respectively, were published. […] PBS criteria in Australia for use of antifibrotics include MDT diagnosis of IPF, HRCT consistent with UIP pattern within 12 months, FVC greater than 50%, FEV1/FVC more than 0.7, DLCO more than 30% and ILD not a result of another known cause. […] Lung transplant remains the only definitive cure for IPF. […] Palliative care is a key part of IPF management and should be addressed at all stages of the disease.

• #54 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.

• #55

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

  Several treatments can help reduce the rate at which IPF gets worse, but there’s currently no treatment that can stop or reverse the scarring of the lungs. […] There’s currently no cure for IPF, but there are several treatments that can help relieve the symptoms and slow down its progression. […] Treatments include: self care measures, such as stopping smoking, eating healthily and exercising regularly; medicines to reduce the rate at which scarring worsens, such as pirfenidone and nintedanib; breathing oxygen through a mask you can do this while you’re at home or while you’re out and about; exercises and advice to help you breathe more easily (pulmonary rehabilitation); a lung transplant this is suitable in a few cases, although donor lungs are rare.

• #56 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.

• #57 IPF (Idiopathic Pulmonary Fibrosis)

  https://www.svhlunghealth.com.au/conditions/ipf-idiopathic-pulmonary-fibrosis

  Currently, there is no cure for IPF. Once present in the lung, scarring cannot be reversed or removed. IPF treatments aim to slow the progression of the disease, manage physical symptoms and improve quality of life. […] Treatment options for IPF include: […] Medicines to slow the scarring and preserve lung function […] Pulmonary rehabilitation includes exercise and education classes to help you conserve energy, exercise safely, manage breathlessness and maximise your quality of life […] Oxygen therapy to boost the oxygen level in your blood and help you breathe […] Lung transplant may be considered in some people […] Clinical Trials. Your doctor may discuss the option of participating in a clinical trial.

• #58 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.

• #59 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. […] Medicines that reduce stomach acid. Gastroesophageal reflux disease, also known as GERD, affects most people with idiopathic pulmonary fibrosis. GERD is linked to worse lung damage. If you have symptoms of acid reflux, your healthcare professional may prescribe ways to treat GERD that reduce stomach acid. […] 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.

• #60 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

  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. There are ongoing clinical trials for PF drugs, including ones to help relieve cough. Visit clinicaltrials.gov to see if any are available near you or ask your doctor.

• #61 Idiopathic pulmonary fibrosis – Wikipedia

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

  Treatment Pulmonary rehabilitation, supplemental oxygen, lung transplantation […] People often benefit from pulmonary rehabilitation and supplemental oxygen. […] Certain medications like pirfenidone or nintedanib may slow the progression of the disease. […] Lung transplantation may also be an option. […] A Cochrane review comparing pirfenidone with placebo, found a reduced risk of disease progression by 30%. […] Nintedanib is a triple angiokinase inhibitor that targets receptor tyrosine kinases involved in the regulation of angiogenesis: fibroblast growth factor receptor (FGFR), platelet-derived growth factor receptor (PDGFR), and vascular endothelial growth factor receptor (VEGFR), which have also been implicated in the pathogenesis of fibrosis and IPF. […] Lung transplantation may be suitable for those patients physically eligible to undergo a major transplant operation. […] Palliative care focuses on reducing symptoms and improving the comfort of patients rather than treating the disease.

• #62 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.

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