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Article Dans Une Revue Rheumatology Année : 2022

Anti-PM-Scl antibodies–positive patients encompass three different groups with distinct prognoses

Luc Mouthon
Yurdagul Uzunhan
  • Fonction : Auteur

Résumé

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.
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Dates et versions

hal-03830880 , version 1 (26-10-2022)

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Paul Breillat, Kuberaka Mariampillai, Paul Legendre, Pauline Martins, Bertrand Dunogue, et al.. Anti-PM-Scl antibodies–positive patients encompass three different groups with distinct prognoses. Rheumatology, 2022, ⟨10.1093/rheumatology/keac508⟩. ⟨hal-03830880⟩
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