You may see patients with connective tissue disorders (CTDs) who develop ILD, including pulmonary fibrosis. Interstitial lung disease (ILD) is a common early manifestation of CTDs which shares common pathogenic pathways to fibrogenesis.2–8

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Improve your ability to diagnose fibrotic ILDs. This atlas can assist you through the process of diagnosing an ILD, helping you recognize key clues and characteristics on chest HRCT and histopathology cuts from surgical lung biopsy.

4 imagining scans, representing fibrotic ILDs and characteristic for them lung pathologies.
  • CTD, connective tissue disease; CTD-ILD, connective tissue disease-associated interstitial lung disease; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; RA, rheumatoid arthritis; RA-ILD, rheumatoid arthritis-associated interstitial lung disease; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease.

  1. Esposito AJ, Chu SG, Madan R, et al. Thoracic manifestations of rheumatoid arthritis. Clin Chest Med. 2019;40(3):545–560.

  2. Fischer A and Distler J. Progressive fibrosing interstitial lung disease associated with systemic autoimmune diseases. Clin Rheumatol. 2019;38(10):2673–2681.

  3. Mathai SC and Danoff SK. Management of interstitial lung disease associated with connective tissue disease. BMJ. 2016;352:h6819.

  4. Wallace B, Vummidi D, Khanna D. Management of connective tissue diseases associated interstitial lung disease: a review of the published literature. Curr Opin Rheumatol. 2016;28(3):236–245.

  5. Spagnolo P, Cordier JF, Cottin V. Connective tissue diseases, multimorbidity and the ageing lung. Eur Respir J. 2016;47(5):1535–1558.

  6. Vacchi C, Sebastiani M, Cassone G, et al. Therapeutic options for the treatment of interstitial lung disease related to connective tissue diseases. A narrative review. J Clin Med. 2020;9(2):407. doi: 10.3390/jcm9020407

  7. Koo SM, Kim SY, Choi SM, et al. Korean guidelines for diagnosis and management of interstitial lung diseases: part 5. Connective tissue disease associated interstitial lung disease. Tuberc Respir Dis (Seoul). 2019;82(4):285–297.

  8. Doyle TJ, Hunninghake GM, Rosas IO. Subclinical Interstitial Lung Disease. Am J Respir Crit Care Med. 2012;185:1147–1153.

  9. Maher TM, Wuyts W. Management of fibrosing interstitial lung diseases. Adv Ther. 2019;36(7):1518–1531.

  10. Hoffmann-Vold AM, Allanore Y, Alves M, et al. Progressive interstitial lung disease in patients with systemic sclerosis- associated interstitial lung disease in the EUSTAR database. Ann Rheum Dis. 2020. Epub ahead of print: doi:10.1136/annrheumdis-2020-217455.

  11. Hoffmann-Vold AM, Maher TM, Philpot EE, et al. The identification and management of interstitial lung disease in systemic sclerosis: evidence-based European consensus statements. Lancet Rheum. 2020;2 e71–e83.

  12. Distler O, Volkmann ER, Hoffmann-Vold AM, et al. Current and future perspectives on management of systemic sclerosis-associated interstitial lung disease. Expert Rev Clin Immunol. 2019;15:1009–1017.

  13. Chowaniec M, Skoczyńska M, Sokolik R, Wiland P. Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management. Reumatologia. 2018;56(4):249–254

  14. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc–ILD). Respir Res. 2019a;20(1):13.

  15. Wells AU, Denton CP. Interstitial lung disease in connective tissue disease—mechanisms and management. Nat Rev Rheumatol. 2014;10:728–739.

  16. Geerts S, Wuyts W, De Langhe E, et al. Connective tissue disease associated interstitial pneumonia: a challenge for both rheumatologists and pulmonologists. Sarcoidosis Vasc Diffuse Lung Dis. 2017;34(4):326–335.

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