PT - JOURNAL ARTICLE AU - Joy, Greta M. AU - Arbiv, Omri A. AU - Wong, Carmen K. AU - Lok, Stacey D. AU - Adderley, Nicola A. AU - Dobosz, Krzysztof M. AU - Johannson, Kerri A. AU - Ryerson, Christopher J. TI - Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis AID - 10.1183/16000617.0210-2022 DP - 2023 Mar 31 TA - European Respiratory Review PG - 220210 VI - 32 IP - 167 4099 - http://err.ersjournals.com/content/32/167/220210.short 4100 - http://err.ersjournals.com/content/32/167/220210.full SO - EUROPEAN RESPIRATORY REVIEW2023 Mar 31; 32 AB - Introduction Interstitial 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.Methods A 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.Results 11 582 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.Discussion We identified substantial variability in ILD across CTD subtypes suggesting that CTD-ILD is too heterogenous to be considered a single entity.Substantial variability exists in interstitial lung disease prevalence, risk factors and computed tomography patterns across connective tissue disease subtypes. Further research is required to better understand the complex pathobiology of this disease. https://bit.ly/3QJXn4d