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Idiopathic disease

IPF

Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia (IIP) (approximately 50% of all IIPs).2 The cause of IPF is unknown.

It is a progressive condition with a poor prognosis and is more common in men, smokers and former smokers. The condition predominantly occurs later in life (>50 years).3

IPF is characterized by a classic UIP pattern: in particular basal and peripheral localized opacities, often accompanied by traction bronchiectasis and ‘honeycombing’.2

man woman

IIP - non-IPF

Besides IPF, idiopathic non-specific interstitial pneumonia (iNSIP) and unclassified IIP are conditions in which fibrosis can occur. Diagnosing IIP is often difficult, due to the heterogeneity of the clinical, radiological and histological findings.2,3

iNSIP accounts for approximately 25% of all IIPs. This diagnosis can only be made when other diagnoses, such as CTD-ILD, drug-induced ILD and familial pulmonary fibrosis, have been excluded. iNSIP is more common in women, people who have never smoked and those over the age of 50.2,3 

An NSIP pattern involves peripheral, symmetrical, and in particular basilar localized ground glass opacities with reticulation (sometimes with subpleural sparing) and traction bronchiectasis. Honeycombing is usually absent.2

Approximately 10-25% of all IIPs cannot be classified.2,3 The clinical picture of unclassified IIP is extremely heterogenous. The combination of clinical, radiological, and pathological findings often does not typically fit with one specific pattern.

References:

  1. Wijsenbeek M, Cottin V. Spectrum of Fibrotic Lung Diseases. N Engl J Med. 2020;383(10):958-68.

  2. Cottin V, Hirani NA, Hotchkin DL, et al. Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150).

  3. Olson AL, Gifford AH, Inase N, et al. The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev. 2018;27(150):180077.

  4. Fischer A, Strek ME, Cottin V, et al. Proceedings of the American College of Rheumatology/Association of Physicians of Great Britain and Ireland Connective Tissue Disease-Associated Interstitial Lung Disease Summit: A Multidisciplinary Approach to Address Challenges and Opportunities. Arthritis Rheumatol. 2019;71(2):182-95.

  5. Vij R, Strek ME. Diagnosis and treatment of connective tissue disease-associated interstitial lung disease. Chest. 2013;143(3):814-24.

  6. Mira-Avendano I, Abril A, Burger CD, et al. Interstitial Lung Disease and Other Pulmonary Manifestations in Connective Tissue Diseases. Mayo Clin Proc. 2019;94(2):309-25.

  7. Kim EJ, Elicker BM, Maldonado F, et al. Usual interstitial pneumonia in rheumatoid arthritis associated interstitial lung disease. Eur Respir J. 2010;35(6):1322-8.

  8. Denton CP, Khanna D. Systemic sclerosis. The Lancet. 2017;390(10103):1685-99.

  9. Nihtyanova SI, Schreiber BE, Ong VH, et al. Prediction of pulmonary complications and long term survival in systemic sclerosis. Arthritis & rheumatology. 2014;66(6):1625-35.

  10. Grunewald J, Grutters JC, Arkema EV, et al. Sarcoidosis. Nat Rev Dis Primers. 2019;5(1):45.

  11. Arkema EV, Cozier YC. Sarcoidosis epidemiology: recent estimates of incidence, prevalence and risk factors. Curr Opin Pulm Med. 2020;26(5):527-34.

  12. ATS Board of Directors, Committee EE. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med. 1999;160(2):736-55.

  13. Ungprasert P, Ryu JH, Matteson EL. Clinical Manifestations, Diagnosis, and Treatment of Sarcoidosis. Mayo Clin Proc Innov Qual Outcomes. 2019;3(3):358-75.

  14. Patterson KC, Strek ME. Pulmonary fibrosis in sarcoidosis. Clinical features and outcomes. Ann Am Thorac Soc. 2013;10(4):362-70.

  15. Raghu G, Remy-Jardin M, Ryerson CJ, et al. Diagnosis of Hypersensitivity Pneumonitis in Adults. An Official ATS/JRS/ALAT Clinical Practice Guideline. Am J Respir Crit Care Med. 2020;202(3):e36-e69.

  16. Spagnolo P, Rossi G, Cavazza A, et al. Hypersensitivity Pneumonitis: A Comprehensive Review. J Investig Allergol Clin Immunol. 2015;25(4):237-50; quiz follow 50.

  17. Spagnolo P, Bonniaud P, Rossi G, et al. Drug-induced interstitial lung disease. Eur Respir J. 2022.