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Hierarchical cluster and survival analyses of antisynthetase syndrome: phenotype and outcome are correlated with anti-tRNA synthetase antibody specificity.
MedLine Citation:
PMID:  22771754     Owner:  NLM     Status:  Publisher    
The clinical phenotype and evolution of antisynthetase syndrome (ASS) are heterogeneous. This study was therefore undertaken to identify subgroups of ASS patients with similar clinico-biological features and outcomes. This retrospective multicentric study included 233 consecutive patients with three different anti-aminoacyl-tRNA-synthetase antibodies (anti-ARS): anti-Jo1 (n=160), anti-PL7 (n=25) & anti-PL12 (n=48). To characterize ASS patients, bivariate, multiple correspondence (MCA), cluster and survival analyses were performed. Interstitial lung disease (ILD) and myositis were the most common ASS manifestations. However, their respective frequencies were correlated to anti-ARS specificity: ILD was more frequent (80% & 88% vs 67%, p=0.014) whereas myositis was less common (44% & 47% vs 74%, p<0.001) in patients with anti-PL7 & anti-PL12 compared to anti-Jo1. The MCA suggested that anti-PL7 & anti-PL12 phenotypes were close to one another and distinct from anti-Jo1. The clustering analysis confirmed these data, identifying subgroups strongly defined by the anti-ARS specificity and other clinical features. Cluster 1 (n=175, 86% of anti-Jo1) defined patients with the most diffuse phenotype, whereas patients from cluster 2 (n=48, 96% of anti-PL12 and anti-PL7) exhibited a disease more restricted to the lung. Patient survival was also conditioned by the anti-ARS specificity, and was significantly lower in patients with anti-PL7/12 rather than anti-Jo1 (p=0.012). Other factors associated with poor survival were mostly related to pulmonary involvement, including severe dyspnea (p=0.003) and isolated ILD (p=0.009) at diagnosis. In patients with ASS, the phenotype and the survival were correlated with the anti-ARS specificity.
Baptiste Hervier; Hervé Devilliers; Raluca Stanciu; Alain Meyer; Yurdagul Uzunhan; Agathe Masseau; Sylvain Dubucquoi; Pierre-Yves Hatron; Lucile Musset; Bruno Wallaert; Hilario Nunes; Thierry Maisonobe; Nils-Olivier Olsson; Daniel Adoue; Philippe Arlet; Jean Sibilia; Marguerite Guiguet; Dominique Lauque; Zahir Amoura; Eric Hachulla; Mohamed Hamidou; Olivier Benveniste
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-7-4
Journal Detail:
Title:  Autoimmunity reviews     Volume:  -     ISSN:  1873-0183     ISO Abbreviation:  -     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-7-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101128967     Medline TA:  Autoimmun Rev     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012. Published by Elsevier B.V.
Internal Medicine Department 2, French Reference Centre for Lupus, Hôpital Pitié-Salpêtrière, APHP, Paris, France & University of Paris VI Pierre & Marie Curie.
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