医学
内科学
背景(考古学)
硬皮病(真菌)
间质性肺病
星团(航天器)
抗体
胃肠病学
肺
病理
免疫学
古生物学
计算机科学
接种
生物
程序设计语言
作者
Paul Breillat,K. Mariampillai,Paul Legendre,Priscila Diniz de Carvalho Martins,Bertrand Dunogué,J.L. Charuel,Makoto Miyara,Claire Goulvestre,Romain Paule,H. Vanquaethem,Félix Ackermann,Olivier Benveniste,Hilario Nunès,Luc Mouthon,Yves Allenbach,Y. Uzunhan
出处
期刊:Rheumatology
[Oxford University Press]
日期:2022-09-05
卷期号:62 (4): 1467-1475
被引量:3
标识
DOI:10.1093/rheumatology/keac508
摘要
To help identify homogeneous subgroups among patients with anti-PM-scleroderma-antibodies (PM-Scl-Abs) positive auto-immune diseases regardless of diagnostic classifications.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.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.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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