Overview
Idiopathic pulmonary fibrosis, often called IPF, is a serious lung disease where the tissue deep inside the lungs becomes scarred and stiff over time. The word 'idiopathic' means doctors do not know the exact cause. As the scarring (called fibrosis) builds up, the lungs lose their ability to stretch and move air in and out properly. This makes it harder and harder for oxygen to pass from the lungs into the bloodstream. IPF is sometimes also referred to as cryptogenic fibrosing alveolitis, especially in older medical records from the United Kingdom. The most common symptoms are a dry, persistent cough and shortness of breath, especially during physical activity. Over time, even simple tasks like walking across a room or climbing stairs can feel exhausting. Some people notice their fingertips or toes become rounded and wider, a sign called 'clubbing.' Fatigue and unintentional weight loss are also common as the disease progresses. There is no cure for IPF, but two medications — nintedanib (Ofev) and pirfenidone (Esbriet) — are approved by the FDA and have been shown to slow the scarring process. Oxygen therapy, pulmonary rehabilitation, and in some cases lung transplantation are also important parts of care. Early diagnosis and treatment give patients the best chance of maintaining quality of life for as long as possible.
Also known as:
Key symptoms:
Shortness of breath, especially during activityDry, persistent cough that does not go awayExtreme tiredness and low energyRounded or widened fingertips and toes (finger clubbing)Unintentional weight lossAching muscles and jointsCrackling sound in the lungs when breathing (heard by a doctor with a stethoscope)Bluish tint to lips or fingertips due to low oxygenDifficulty sleeping due to breathing problemsReduced ability to exercise or do physical tasks
Clinical phenotype terms (20)— hover any for plain English
Multifactorial
Caused by a mix of several genes and environmental factors
Late onset
Begins later in life, typically after age 50
FDA & Trial Timeline
10 eventsBristol-Myers Squibb — PHASE3
University of Modena and Reggio Emilia
Haisco Pharmaceutical Group Co., Ltd. — PHASE3
NINTEDANIB: FDA approved
NINTEDANIB: FDA approved
NINTEDANIB: FDA approved
Dragonboat Biopharmaceutical Company Limited — PHASE2
Hubei Bio-Pharmaceutical Industrial Technological Institute Inc. — PHASE1
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
4 availablePirfenidone Capsule, 267 Mg
Pirfenidone capsules are indicated for the treatment of idiopathic pulmonary fibrosis (IPF)
Jascayd
treatment of idiopathic pulmonary fibrosis (IPF) in adult patients
Ofev
Treatment of idiopathic pulmonary fibrosis
Pirfenidone
Treatment of idiopathic pulmonary fibrosis
Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Financial Resources
3 resourcesTravel Grants
No travel grants are currently matched to Idiopathic pulmonary fibrosis.
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2 articlesCaregiver Resources
NORD Caregiver Resources
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Mental Health Support
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Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.Which antifibrotic medication do you recommend for me, and what side effects should I watch out for?,How quickly is my lung function declining, and how will we track this over time?,Should I be referred for pulmonary rehabilitation, and is it available near me?,At what point should we start discussing lung transplantation as an option?,Do I need supplemental oxygen now, or should I be tested for low oxygen during activity or sleep?,Given my family history, should my relatives be screened or tested for genetic risk factors?,Are there any clinical trials I might be eligible for?
Common questions about Idiopathic pulmonary fibrosis
What is Idiopathic pulmonary fibrosis?
Idiopathic pulmonary fibrosis, often called IPF, is a serious lung disease where the tissue deep inside the lungs becomes scarred and stiff over time. The word 'idiopathic' means doctors do not know the exact cause. As the scarring (called fibrosis) builds up, the lungs lose their ability to stretch and move air in and out properly. This makes it harder and harder for oxygen to pass from the lungs into the bloodstream. IPF is sometimes also referred to as cryptogenic fibrosing alveolitis, especially in older medical records from the United Kingdom. The most common symptoms are a dry, persiste
How is Idiopathic pulmonary fibrosis inherited?
Idiopathic pulmonary fibrosis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Idiopathic pulmonary fibrosis typically begin?
Typical onset of Idiopathic pulmonary fibrosis is late onset. Age of onset can vary across affected individuals.
Are there clinical trials for Idiopathic pulmonary fibrosis?
Yes — 20 recruiting clinical trials are currently listed for Idiopathic pulmonary fibrosis on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Idiopathic pulmonary fibrosis?
25 specialists and care centers treating Idiopathic pulmonary fibrosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Idiopathic pulmonary fibrosis?
3 patient support programs are currently tracked on UniteRare for Idiopathic pulmonary fibrosis. See the treatments and support programs sections for copay assistance, eligibility, and contact details.