Idiopathic Pulmonary Fibrosis
Idiopathic Pulmonary Fibrosis
Also known as:IPF; usual interstitial pneumonia; China First Rare Disease Catalog item 55
Idiopathic pulmonary fibrosis is a progressive interstitial lung disease of unknown cause characterized by scarring of lung tissue, manifesting as exertional dyspnea and dry cough; antifibrotic medications can slow decline, and lung transplantation is the only cure.
Start Here
A quick guide to the next step: which department to start with, what to prepare, and what to ask.
Pulmonology or an interstitial lung disease specialty clinic for progressive exertional dyspnea and persistent dry cough, especially in men over 60.
A chronic, progressive, and irreversible interstitial lung disease where lung tissue becomes scarred (fibrotic), reducing elasticity and impairing oxygen exchange.
Antifibrotic medications (pirfenidone, nintedanib) slow lung function decline; nerandomilast was FDA-approved in 2025. Lung transplantation is the only curative option.
Most cases are sporadic; approximately 20% have a family history, associated with telomerase gene (TERT, TERC) mutations. Smoking and certain environmental exposures are risk factors.
Early symptoms (dyspnea, dry cough) are often attributed to aging, chronic bronchitis, or asthma; diagnosis is frequently delayed by more than a year.
Common Search and Care Questions
This page helps patients and families organize care leads. It does not replace a clinician’s diagnosis or treatment plan. For testing, medication, referrals, emergency care, and support applications, follow qualified clinicians, medical institutions, support organizations, and official sources.
Diagnosis Path
Organized around the practical patient journey: identify clues, avoid common delays, then prepare for care.
When to Suspect It
- Progressive exertional dyspnea, especially with walking or climbing stairs.
- Persistent dry cough unresponsive to cough suppressants.
- Fine bibasilar crackles (Velcro rales) on lung auscultation.
- Digital clubbing.
Common Wrong Turns
- Attributing dyspnea to "getting older" or poor fitness.
- Treating dry cough as chronic pharyngitis or bronchitis for months.
- Repeated normal chest X-rays at primary care without high-resolution CT.
- Inappropriate use of corticosteroids or immunosuppressants, which can be harmful in IPF.
Departments to Start With
- Pulmonology (interstitial lung disease clinic)
- Thoracic surgery / lung transplant evaluation (moderate-to-severe disease)
- Radiology (HRCT assessment)
- Rehabilitation medicine (pulmonary rehabilitation)
Before the Visit
- Document symptom duration, progression, and smoking/environmental exposure history.
- High-resolution CT (HRCT): look for peripheral, basal-predominant reticulation and honeycombing.
- Pulmonary function tests: restrictive pattern with reduced DLCO.
- Exclude other interstitial lung diseases (connective tissue disease, hypersensitivity pneumonitis, drug-induced ILD).
- Surgical lung biopsy if HRCT is indeterminate.
Tests to Ask About
- High-resolution chest CT (HRCT).
- Pulmonary function tests (FVC, DLCO).
- 6-minute walk test.
- Autoantibody panel (exclude connective tissue disease-associated ILD).
- Bronchoalveolar lavage (if needed).
Questions for the Doctor
- Does my CT show a definite UIP pattern? Is a biopsy needed?
- Am I a candidate for pirfenidone or nintedanib? How are side effects managed?
- How fast is my lung function declining?
- When should I be evaluated for lung transplantation?
- How is an acute exacerbation managed?
Basic Information
Medical Notes
More complete medical explanations are kept here for discussion with clinicians.
Symptoms
Typical presentation is insidious onset of progressive exertional dyspnea and persistent dry cough. Some patients have fatigue, weight loss, and arthralgia. Physical examination commonly reveals fine bibasilar crackles (Velcro rales) and digital clubbing. Advanced disease leads to severe hypoxemia, pulmonary hypertension, and right heart failure. Acute exacerbations present as rapid worsening of dyspnea over days to weeks and carry a high mortality.
Diagnosis
Diagnosis relies on clinical presentation, HRCT, and exclusion of other causes. Definite UIP pattern on HRCT includes peripheral and basal-predominant reticulation, traction bronchiectasis, and honeycombing. If HRCT shows possible UIP or inconsistent with UIP, surgical lung biopsy or multidisciplinary discussion (MDD) is needed. Pulmonary function tests show restrictive impairment with reduced DLCO. It is essential to exclude connective tissue disease, chronic hypersensitivity pneumonitis, asbestosis, and drug-induced lung disease.
Treatment
Antifibrotic therapy with pirfenidone and nintedanib slows the rate of FVC decline and is recommended for mild-to-moderate disease. In October 2025, the FDA approved nerandomilast (a PDE4B inhibitor), which reduced annual FVC decline by 52.5% in phase 3 trials. Supportive care includes smoking cessation, oxygen therapy, pulmonary rehabilitation, influenza and pneumococcal vaccination, and gastroesophageal reflux management. Acute exacerbations are treated with supportive care and corticosteroids (evidence is limited). Lung transplantation should be considered early for patients with progressive decline. Routine corticosteroids, immunosuppressants, and N-acetylcysteine are not recommended.
Long-term Care
Follow-up every 3–6 months with pulmonary function tests, 6-minute walk distance, and symptom assessment. The GAP score (gender, age, FVC, DLCO) helps estimate prognosis. Monitor drug adverse effects (pirfenidone: rash, photosensitivity, GI upset; nintedanib: diarrhea, weight loss, elevated liver enzymes). Pulmonary rehabilitation improves exercise capacity and quality of life. Avoid smoking and known environmental exposures.
Fertility and Family
A minority of patients have familial IPF, associated with telomere-related gene mutations. Those with a family history may consider genetic counseling and testing. Family planning should be managed jointly by pulmonology and obstetrics, assessing whether pulmonary function can tolerate pregnancy.
When to Seek Urgent Care
Sudden severe dyspnea, worsening hypoxemia, fever with productive cough (possible acute exacerbation or infection), hemoptysis, or altered mental status require immediate emergency care.
Prognosis
Median survival 3–5 years; antifibrotics slow progression; acute exacerbations have poor prognosis; early diagnosis and treatment improve quality of life.