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Underlaying systemic disease

CTD-ILD

All patients with connective tissue disease are at risk for developing ILD.4 At particularly high risk to develop pulmonary fibrosis are patients with systemic sclerosis (SSc) and polymyositis/dermatomyositis (PM/DM). Patients with rheumatoid arthritis (RA), representing a large group within the CTDs, may also develop pulmonary fibrosis. Fibrosis can also occur in Sjögren’s syndrome and systemic lupus erythematosus.4 Sometimes, the ILD manifests earlier than the CTD, making diagnosis difficult.5,6

In RA-ILD, a UIP pattern is frequently observed.1,7 [see IPF for explanation of UIP pattern] In SSc-ILD, fibrosis of the skin and other organs, including the lungs, can occur. Usually, an NSIP pattern is seen in the lungs.2,3,8 [see IIP (non-IPF) for explanation of NSIP pattern.] A distinction is made between a limited and diffuse form of SSc. In the diffuse form, clinically significant pulmonary fibrosis is more common and manifests more rapidly than in the limited form.9

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Sarcoidosis

Sarcoidosis is a multisystem inflammatory disease of unknown cause. An exaggerated immune response to an unknown trigger occurs in genetically predisposed individuals.2 Sarcoidosis usually first presents in those between 20 and 50 years of age with a second peak in postmenopausal women.10-13 

Sarcoidosis appears to be slightly more common in women than in men. The majority of patients with sarcoidosis have lung involvement. Although most patients go into remission, up to 20% of patients develop pulmonary fibrosis as a response to inflammation.14 The pattern of fibrosis in the lungs is extremely variable.

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.