"To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity.

Abstract

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, Alarcón-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, Alarcón-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 Alarcón-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.

• Authors: Susanna Cappelli, Silvia Bellando Randone, Dušanka Martinović, Maria-Magdalena Tamas, Katarina Pasalić, Yannick Allanore, Marta Mosca, Rosaria Talarico, Daniela Opris, Csaba G Kiss, Anne-Kathrin Tausche, Silvia Cardarelli, Valeria Riccieri, Olga Koneva, Giovanna Cuomo, Mike Oliver Becker, Alberto Sulli, Serena Guiducci, Mislav Radić, Stefano Bombardieri, Martin Aringer, Franco Cozzi, Guido Valesini, Lidia Ananyeva, Gabriele Valentini, Gabriela Riemekasten, Maurizio Cutolo, Ruxandra Ionescu, László Czirják, Nemanja Damjanov, Simona Rednic, Marco Matucci Cerinic
• Journal: Seminars in arthritis and rheumatism (Semin Arthritis Rheum) Vol. 41 Issue 4 Pg. 589-98 (Feb 2012) ISSN: 1532-866X [Electronic] United States
• PMID: 21959290 (Publication Type: Journal Article, Multicenter Study)
• Copyright: Copyright © 2012 Elsevier Inc. All rights reserved.

Chemical References

• Autoantibodies

Topics

• Adult
• Autoantibodies (immunology)
• Disease Progression
• Female
• Follow-Up Studies
• Humans
• Male
• Mixed Connective Tissue Disease (classification, diagnosis, immunology)
• Retrospective Studies