Idiopathic pulmonary fibrosis is a scarring disease of the lungs with no known cause that mainly affects people over 60, often former smokers. The scar tissue stiffens the lungs and slowly lowers oxygen, so early diagnosis and antifibrotic drugs matter.
Key symptoms
- Shortness of breath that slowly worsens over months
- Dry, persistent cough
- Fatigue and weight loss
- Breathlessness that limits daily activities
What you find on exam
- Fine, dry velcro-like crackles at both lung bases
- Digital clubbing
- Oxygen saturation at rest and after walking
- Signs of right heart strain in late disease
Tests
- High-resolution CT: honeycombing and scarring at the bases (UIP pattern)
- Pulmonary function tests: restrictive pattern and low DLCO
- Six-minute walk test with oximetry
- Autoimmune tests and exposure history to rule out other causes
Treatment basics
- Antifibrotic drugs: nintedanib or pirfenidone
- Oxygen for low saturation
- Pulmonary rehabilitation and vaccines
- Treat acid reflux; stop smoking
- Early referral for lung transplant evaluation
Red flags
- Rapid worsening of breathlessness over days (acute exacerbation)
- Low oxygen saturation at rest
- Signs of right heart failure
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Sources: NCCPA PANCE Content Blueprint; NBOME COMLEX-USA clinical presentations; AAMC Core Entrustable Professional Activities (Core EPAs). Written by Making Synapses. Making Synapses is not affiliated with these organizations.