LAUSR

OBJECTIVE To help identify homogeneous subgroups among patients with anti-polymyositis-scleroderma-antibodies (PM-Scl-Abs) positive auto-immune diseases regardless of diagnostic classifications. METHODS This multicentric (four hospitals) retrospective study collected all consecutive patients (from 2011 to 2021) with positive testing for anti-PM-Scl-Abs in a context of connective tissue disease. Subgroups of patients with similar clinico-biological phenotypes were defined using unsupervised multiple correspondence analysis and hierarchical clustering analysis of the features recorded in the first year of follow-up. RESULTS One hundred and forty-two patients with anti-PM-Scl-Abs were evaluated and 129 patients were included in the clustering analysis and divided into 3 clusters. Cluster n°1 (n = 47) included patients with frequent skin thickening, digestive involvement and interstitial lung disease (ILD) with Non Specific Interstitial Pneumonia (NSIP). They were more likely to develop progressive fibrosing ILD. Cluster n°2 (n = 36) included patients who all featured NSIP with frequent Organizing pneumonia associated pattern and mechanic's hands. This subgroup had increased risk of relapse and ILD was characterized by a good functional outcome. Cluster n°3 (n = 46) was characterized by predominant or isolated musculoskeletal involvement and frequently matched undifferentiated connective tissue disease (UCTD) criteria. Although very frequent among Anti PM-Scl-Abs positive patients, muscle involvement was less discriminating compared with skin thickening and ILD pattern to classify patients into subgroups. CONCLUSION Anti-PM-Scl-Abs associated auto-immune diseases are segregated into 3 subgroups with distinct clinical phenotype and outcomes. Skin thickening and NSIP are determinant predictors in segregation of theses populations.