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.
|Autori:||Cappelli S; Bellando Randone S; Martinovic D; Tamas MM; Pasalic K; Allanore Y; Mosca M; Talarico R; Opris D; Kiss CG; Tausche AK; Cardarelli S; Riccieri V; Koneva O; Cuomo G; Becker MO; Sulli A; Guiducci S; Radic M; Bombardieri S; Aringer M; Cozzi F; Valesini G; Ananyeva L; Valentini G; Riemekasten G; Cutolo M; Ionescu R; Czirják L; Damjanov N; Rednic S; Matucci Cerinic M.|
|Titolo:||"To be or not to be," ten years after: evidence for mixed connective tissue disease as a distinct entity.|
|Anno del prodotto:||2012|
|Appare nelle tipologie:||1.1 Articolo in rivista|