Interstitial Lung Disease is not one disease, it’s an umbrella term for more than 200 conditions that affect the tissue between the air sacs of the lungs.
The common thread is that the lung tissue is becoming scarred. Scarred lung does not stretch or transfer oxygen normally. Over time, the lungs become small, stiff, and inefficient. Roughly 200,000 Americans have IPF, and over 600,000 have some form of ILD. Newer antifibrotic therapies have changed prognosis materially.
Interstitial Lung Disease is not one disease, it’s an umbrella term for more than 200 conditions that affect the tissue between the air sacs of the lungs (the interstitium). The most common forms in adults are idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis, connective-tissue-disease-associated ILD (from rheumatoid arthritis, scleroderma, etc.), and drug-induced ILD. The common thread is that the lung tissue is becoming scarred.
Progressive shortness of breath on exertion is almost universal. Dry cough is common. Many patients develop “Velcro crackles” on lung exam, a distinctive crackling sound during inhalation. Finger clubbing (rounded, bulbous fingertips) develops in advanced disease. By the time most patients arrive at a pulmonologist, they’ve had symptoms for 1–2 years and have been told they have “asthma” or “deconditioning.”
Diagnosis requires three things: history (occupational, medication, family, autoimmune), high-resolution CT chest, and often pulmonary function testing and labs. The HRCT pattern often gives the diagnosis. The classic IPF pattern is “usual interstitial pneumonia (UIP)” with subpleural reticulation and honeycombing. In ambiguous cases, multi-disciplinary discussion (pulmonologist, radiologist, sometimes pathologist) is the gold standard. Lung biopsy is reserved for cases where the answer materially changes management.
Treatment depends on the type of ILD. IPF: antifibrotic therapy with nintedanib (Ofev) or pirfenidone (Esbriet), both reduce annual lung function decline by roughly half. Hypersensitivity pneumonitis: remove the trigger; corticosteroids in some cases. CTD-ILD: treat the underlying connective tissue disease. Drug-induced: stop the offending drug. Across all types, supportive care includes pulmonary rehabilitation, oxygen for hypoxia, vaccination, anti-reflux therapy, and early referral for lung transplant evaluation in progressive disease.
This page is general medical information, not personalized medical advice. If you have questions about your specific health, talk with your Nimbus clinician.
