Open AccessDetection and Management of Interstitial Lung Diseases Associated With Connective Tissue Diseases
Author(s) -
Castelino Flavia V.,
Moua Teng
Publication year - 2021
Publication title -
acr open rheumatology
Language(s) - English
Resource type - Journals
ISSN - 2578-5745
DOI - 10.1002/acr2.11253
Subject(s) - medicine , pulmonologists , nintedanib , interstitial lung disease , ctd , connective tissue disease , connective tissue , lung , high resolution computed tomography , idiopathic pulmonary fibrosis , pathology , intensive care medicine , radiology , disease , autoimmune disease , oceanography , geology
Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs). A proportion of patients with CTD‐ILDs develop progressive fibrosing ILD, which is characterized by worsening fibrotic abnormalities on high‐resolution computed tomography scan, decline in lung function, worsening symptoms, and early mortality. Here, we review the impact of ILD in patients with CTDs, the importance of prompt diagnosis and close monitoring, and the evidence available to guide the management of CTD‐ILDs. Management of patients with CTD‐ILDs should be individualized and involve close collaboration between rheumatologists and pulmonologists. Immunosuppression is the mainstay of therapy for CTDs, but evidence for its effectiveness in slowing the progression of ILD is limited. Recently, nintedanib has been approved to slow decline in lung function in patients with systemic sclerosis–associated ILD and chronic fibrosing ILDs with a progressive phenotype. The results of ongoing clinical trials will help clinicians take a more evidence‐based approach to the treatment of CTD‐ILDs.