Interstitial lung diseases (ILD) encompass a broad group of more than 200 parenchymal pulmonary disorders in which the lung tissue is comprised. Connective tissue disease related ILD comprise almost 20% of all ILD, rheumatoid arthritis and systemic sclerosis (SSc) making up the largest proportion of CTD-ILD.1 Some CTD-ILD may have an acute course, such as anti-MDA5 positive rapidly progressive amyopathic dermatomyositis related ILD, others may have a chronic course. CTD-ILD are frequently progressive bringing along an as dismal disease course as idiopathic pulmonary fibrosis. Several challenges are related to CTD-ILD. One of the challenges concerning is firstly to think of them. The second challenge is to screen for them. The third challenge is the optimal management. In this way only for SSc related ILD we have generally adopted screening/monitoring strategies as well as several randomised controlled trials to evaluate the efficacy of immune suppressive and antifibrotic drugs.2 Multidisciplinary approach in diagnosis and management of CTD-ILD is paramount.
Wijsenbeek M, et al. Interstitial lung diseases. Lancet. 2022 Sep 3;400(10354):769–786. doi: 10.1016/S0140-6736(22)01052-2. Epub 2022 Aug 11.
Volkmann ER, et al. Systemic sclerosis. Lancet. 2023 Jan 28;401(10373):304–318. doi: 10.1016/S0140-6736(22)01692-0. Epub 2022 Nov 25.
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