IntroductionInterstitial lung disease (ILD) is a frequent manifestation of connective tissue disease (CTD) with substantial variability in prevalence and outcomes reported across CTD subtypes. This systematic review summarises the prevalence, risk factors and ILD patterns on chest computed tomography of CTD-ILD.MethodsA comprehensive search was performed in Medline and Embase to identify eligible studies. Meta-analyses were completed using a random effects model to determine the pooled prevalence of CTD-ILD and ILD patterns.Results11 582 unique citations were identified with 237 articles included. Pooled prevalence of ILD was 11% in rheumatoid arthritis (95% CI 7–15%), 47% in systemic sclerosis (44–50%), 41% in idiopathic inflammatory myositis (33–50%), 17% in primary Sjögren's syndrome (12–21%), 56% in mixed connective tissue disease (39–72%) and 6% in systemic lupus erythematosus (3–10%). Usual interstitial pneumonia was the most prevalent ILD pattern in rheumatoid arthritis (pooled prevalence of 46%), while nonspecific interstitial pneumonia was the most common ILD pattern in all other CTD subtypes (pooled prevalence range 27–76%). Across all CTDs with available data, positive serology and higher inflammatory markers were risk factors for development of ILD.DiscussionWe identified substantial variability in ILD across CTD subtypes suggesting that CTD-ILD is too heterogenous to be considered a single entity.

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