¡°To Be or Not To Be,?Ten Years After: Evidence for Mixed Connective Tissue Disease as a Distinct Entity

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• 作者：Susanna Cappelli ; MD^?/sup> ; ^{susicappelli82@yahoo.it} ; Silvia Bellando Randone ; MD^?/sup> ; Duš ; anka Martinović ; MD ; PhD^&dagger ; ; Maria-Magdalena Tamas ; MD^&Dagger ; ; Katarina Pasalić ; MD^§ ; ; Yannick Allanore ; MD ; PhD^¶ ; ; Marta Mosca ; MD ; PhD^?/sup> ; Rosaria Talarico ; MD ; PhD^?/sup> ; Daniela Opris ; MD^?? ; Csaba G. Kiss ; MD ; PhD^&dagger ; &dagger ; ; Anne-Kathrin Tausche ; MD^&Dagger ; &Dagger ; ; Silvia Cardarelli ; MD^§ ; § ; ; Valeria Riccieri ; MD ; PhD^¶ ; ¶ ; ; Olga Koneva ; MD^¡Î¡Î ; Giovanna Cuomo ; MD ; PhD^???/sup> ; Mike Oliver Becker ; MD^&dagger ; &dagger ; &dagger ; ; Alberto Sulli ; MD^&Dagger ; &Dagger ; &Dagger ; ; Serena Guiducci ; MD ; PhD^?/sup> ; Mislav Radić ; MD^&dagger ; ; Stefano Bombardieri ; MD^?/sup> ; Martin Aringer ; MD ; PhD^&Dagger ; &Dagger ; ; Franco Cozzi ; MD^§ ; § ; ; Guido Valesini ; MD^¶ ; ¶ ; ; Lidia Ananyeva ; MD^¡Î¡Î ; Gabriele Valentini ; MD^???/sup> ; Gabriela Riemekasten ; MD ; PhD^&dagger ; &dagger ; &dagger ; ; Maurizio Cutolo ; MD ; PhD^&Dagger ; &Dagger ; &Dagger ; ; Ruxandra Ionescu ; MD ; PhD^?? ; Lá ; szló ; Czirjá ; k ; MD ; PhD^&dagger ; &dagger ; ; Nemanja Damjanov ; MD ; PhD^§ ; ; Simona Rednic ; MD ; PhD^&Dagger ; ; Marco Matucci Cerinic ; MD ; PhD^?/sup>
• 关键词：mixed connective tissue disease ; systemic sclerosis ; systemic lupus erythematosus ; rheumatoid arthritis ; overlap syndrome ; undifferentiated connective tissue disease ; autoantibodies
• 刊名：Seminars in Arthritis and Rheumatism
• 出版年：2012
• 出版时间：February 2012
• 年：2012
• 卷：41
• 期：4
• 页码：589-598
• 全文大小：288 K

文摘

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Objectives

To determine if mixed connective tissue disease (MCTD) can be considered an independent clinical entity, to compare 3 different classification criteria for MCTD (Kasukawa, Alarc¨®n-Segovia, and Sharp), and to define predictors (clinical features and autoantibodies) of potential evolution toward other connective tissue diseases (CTDs).

Methods

One hundred sixty-one MCTD patients were evaluated retrospectively at the diagnosis and in 2008. They were classified, at the diagnosis, according to the 3 classification criteria of MCTD (Sharp, Alarc¨®n-Segovia, and Kasukawa) and reclassified in 2008 according to their evolution. Statistical analyses were performed to find out predictors (clinical features and autoantibodies) of evolution into other CTDs.

Results

After a mean of 7.9 years of disease, 57.9 % of patients still satisfied MCTD classification criteria of Kasukawa; 17.3 % evolved into systemic sclerosis, 9.1 % into systemic lupus erythematosus, 2.5 % into rheumatoid arthritis, 11.5 % was reclassified as affected by undifferentiated connective tissue disease, and 1.7 % as suffering from overlap syndrome. Kasukawa's criteria were more sensitive (75 % ) in comparison to those of Alarc¨®n-Segovia (73 % ) and Sharp (42 % ). The presence of anti-DNA antibodies (P = 0.012) was associated with evolution into systemic lupus erythematosus; hypomotility or dilation of esophagus (P < 0.001); and sclerodactyly (P = 0.034) with evolution into systemic sclerosis.

Conclusions

MCTD is a distinct clinical entity but it is evident that a subgroup of patients may evolve into another CTD during disease progression. Initial clinical features and autoantibodies can be useful to predict disease evolution.