Respiratory and sleep medicine
Interstitial lung disease
Classifying ILD, the HRCT patterns and multidisciplinary diagnosis of IPF, progressive pulmonary fibrosis, antifibrotics and their PBS access, nerandomilast, acute exacerbations and the classic traps (triple therapy, upper versus lower zone, drug causes).
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- ILD is a group of more than 200 disorders that inflame or scar the lung interstitium. Most present with exertional breathlessness, dry cough and fine basal crackles.
- Idiopathic pulmonary fibrosis (IPF) is the commonest idiopathic type. It affects older men and ex-smokers, and untreated median survival is about 3 to 5 years.
- The key test is HRCT. Diagnosis is made at an ILD multidisciplinary meeting (respiratory physician, radiologist, pathologist, often a rheumatologist).
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Sources
- Raghu G et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2022;205:e18-e47American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, Latin American Thoracic Association, 2022
- Raghu G et al. Diagnosis of hypersensitivity pneumonitis in adults: an official ATS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2020;202:e36-e69American Thoracic Society, Japanese Respiratory Society, Latin American Thoracic Association, 2020
- Antoniou KM, Distler O et al. ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. Eur Respir J 2026European Respiratory Society and EULAR, 2026
- Richeldi L et al. Nerandomilast in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF). N Engl J Med 2025;392:2193-2202New England Journal of Medicine, 2025
- Maher TM et al. Nerandomilast in patients with progressive pulmonary fibrosis (FIBRONEER-ILD). N Engl J Med 2025;392:2203-2214New England Journal of Medicine, 2025
- Brown MB. Nerandomilast: first approval. Drugs 2026Drugs (Springer), 2026
- Services Australia: nintedanib for progressive fibrosing interstitial lung disease, PBS authorityAU/NZServices Australia, accessed November 2026
- Pharmaceutical Benefits Scheme: nintedanib and pirfenidone listingsAU/NZAustralian Government Department of Health, Disability and Ageing, online schedule, accessed November 2026
- Naccache JM et al. Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP). Lancet Respir Med 2022;10(1):26-34The Lancet Respiratory Medicine, 2022
- JASCAYD (nerandomilast) US prescribing informationBoehringer Ingelheim (DailyMed), label updated 14 August 2026
- Khor YH et al. Acute exacerbation in fibrotic interstitial lung disease: an international working group report. Am J Respir Crit Care Med 2026American Journal of Respiratory and Critical Care Medicine, 2026 (abstract read)
- Idiopathic Pulmonary Fibrosis Clinical Trials Group (PANTHER-IPF). Prednisone, azathioprine and N-acetylcysteine for pulmonary fibrosis. N Engl J Med 2012;366:1968-77New England Journal of Medicine, 2012
- Flaherty KR et al. Nintedanib in progressive fibrosing interstitial lung diseases (INBUILD). N Engl J Med 2019;381:1718-27New England Journal of Medicine, 2019
- Last updated
- 14 November 2026
What changed
- 14 November 2026 Personal emails, About story and Australian-style copy