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.
Untreated IPF patients lose roughly 220 mL of FVC per year; on antifibrotic therapy, that loss is approximately 110 mL per year — about half. Over 5 years, this difference is the difference between meaningful exercise capacity and oxygen dependence.
INPULSIS-1 and -2 reported nintedanib reduced annual FVC decline by ~110 mL versus placebo. ASCEND pooled with CAPACITY showed pirfenidone reduced ≥10% FVC decline by ~48%.
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.
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.
