Anti-PM-Scl antibodies–positive patients encompass three different groups with distinct prognoses. (5th September 2022)

• Record Type: Journal Article
• Authors: Breillat, Paul; Mariampillai, Kuberaka; Legendre, Paul; Martins, Pauline; Dunogue, Bertrand; Charuel, Jean Luc; Miyara, Makoto; Goulvestre, Claire; Paule, Romain; Vanquaethem, Helene; Ackermann, Felix; Benveniste, Olivier; Nunes, Hilario; Mouthon, Luc; Allenbach, Yves; Uzunhan, Yurdagul
• Is Part Of: Rheumatology. Volume 62:Number 4(2023)
• Page Start: 1467
• Page End: 1475
• Publication Date: 2022-09-05
• Subjects: SSc -- idiopathic inflammatory myopathies -- anti-PM-Scl antibodies -- cluster study; Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/keac508
• Languages: English
• ISSNs: 1462-0324

Abstract

Abstract: Objective: To help identify homogeneous subgroups among patients with anti-PM-scleroderma-antibodies (PM-Scl-Abs) positive auto-immune diseases regardless of diagnostic classifications. Material and 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 CTD. 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 three clusters. Cluster 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 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 3 ( n = 46) was characterized by predominant or isolated musculoskeletal involvement and frequently matched 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 three subgroups with distinct clinical phenotype and outcomes. Skin thickening and NSIP are determinant predictors in segregation of theses populations.