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

Susanna Cappelli, Silvia Bellando Randone, Dušanka Martinović, Maria Magdalena Tamas, Katarina Pasalić, Yannick Allanore, Marta Mosca, Rosaria Talarico, Daniela Opris, C. Kiss, Anne Kathrin Tausche, Silvia Cardarelli, Valeria Riccieri, Olga Koneva, Giovanna Cuomo, Mike Oliver Becker, Alberto Sulli, Serena Guiducci, Mislav Radić, Stefano BombardieriMartin Aringer, Franco Cozzi, Guido Valesini, Lidia Ananyeva, Gabriele Valentini, Gabriela Riemekasten, Maurizio Cutolo, Ruxandra Ionescu, L. Czirják, Nemanja Damjanov, Simona Rednic, Marco Matucci Cerinic

Research output: Contribution to journalArticle

71 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)589-598
Number of pages10
JournalSeminars in Arthritis and Rheumatism
Volume41
Issue number4
DOIs
Publication statusPublished - Feb 2012

Fingerprint

Mixed Connective Tissue Disease
Connective Tissue Diseases
Autoantibodies
Systemic Scleroderma
Systemic Lupus Erythematosus
Antinuclear Antibodies
Esophagus
Disease Progression
Dilatation
Rheumatoid Arthritis

Keywords

  • Autoantibodies
  • Mixed connective tissue disease
  • Overlap syndrome
  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Systemic sclerosis
  • Undifferentiated connective tissue disease

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine

Cite this

Cappelli, S., Bellando Randone, S., Martinović, D., Tamas, M. M., Pasalić, K., Allanore, Y., ... Matucci Cerinic, M. (2012). "To be or not to be," ten years after: Evidence for mixed connective tissue disease as a distinct entity. Seminars in Arthritis and Rheumatism, 41(4), 589-598. https://doi.org/10.1016/j.semarthrit.2011.07.010

"To be or not to be," ten years after : Evidence for mixed connective tissue disease as a distinct entity. / Cappelli, Susanna; Bellando Randone, Silvia; Martinović, Dušanka; Tamas, Maria Magdalena; Pasalić, Katarina; Allanore, Yannick; Mosca, Marta; Talarico, Rosaria; Opris, Daniela; Kiss, C.; Tausche, Anne Kathrin; Cardarelli, Silvia; Riccieri, Valeria; Koneva, Olga; Cuomo, Giovanna; Becker, Mike Oliver; Sulli, Alberto; Guiducci, Serena; Radić, Mislav; Bombardieri, Stefano; Aringer, Martin; Cozzi, Franco; Valesini, Guido; Ananyeva, Lidia; Valentini, Gabriele; Riemekasten, Gabriela; Cutolo, Maurizio; Ionescu, Ruxandra; Czirják, L.; Damjanov, Nemanja; Rednic, Simona; Matucci Cerinic, Marco.

In: Seminars in Arthritis and Rheumatism, Vol. 41, No. 4, 02.2012, p. 589-598.

Research output: Contribution to journalArticle

Cappelli, S, Bellando Randone, S, Martinović, D, Tamas, MM, Pasalić, K, Allanore, Y, Mosca, M, Talarico, R, Opris, D, Kiss, C, Tausche, AK, Cardarelli, S, Riccieri, V, Koneva, O, Cuomo, G, Becker, MO, Sulli, A, Guiducci, S, Radić, 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 2012, '"To be or not to be," ten years after: Evidence for mixed connective tissue disease as a distinct entity', Seminars in Arthritis and Rheumatism, vol. 41, no. 4, pp. 589-598. https://doi.org/10.1016/j.semarthrit.2011.07.010
Cappelli, Susanna ; Bellando Randone, Silvia ; Martinović, Dušanka ; Tamas, Maria Magdalena ; Pasalić, Katarina ; Allanore, Yannick ; Mosca, Marta ; Talarico, Rosaria ; Opris, Daniela ; Kiss, C. ; Tausche, Anne Kathrin ; Cardarelli, Silvia ; Riccieri, Valeria ; Koneva, Olga ; Cuomo, Giovanna ; Becker, Mike Oliver ; Sulli, Alberto ; Guiducci, Serena ; Radić, Mislav ; Bombardieri, Stefano ; Aringer, Martin ; Cozzi, Franco ; Valesini, Guido ; Ananyeva, Lidia ; Valentini, Gabriele ; Riemekasten, Gabriela ; Cutolo, Maurizio ; Ionescu, Ruxandra ; Czirják, L. ; Damjanov, Nemanja ; Rednic, Simona ; Matucci Cerinic, Marco. / "To be or not to be," ten years after : Evidence for mixed connective tissue disease as a distinct entity. In: Seminars in Arthritis and Rheumatism. 2012 ; Vol. 41, No. 4. pp. 589-598.
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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, Alarc{\'o}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{\'o}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{\'o}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.",
keywords = "Autoantibodies, Mixed connective tissue disease, Overlap syndrome, Rheumatoid arthritis, Systemic lupus erythematosus, Systemic sclerosis, Undifferentiated connective tissue disease",
author = "Susanna Cappelli and {Bellando Randone}, Silvia and Dušanka Martinović and Tamas, {Maria Magdalena} and Katarina Pasalić and Yannick Allanore and Marta Mosca and Rosaria Talarico and Daniela Opris and C. Kiss and Tausche, {Anne Kathrin} and Silvia Cardarelli and Valeria Riccieri and Olga Koneva and Giovanna Cuomo and Becker, {Mike Oliver} and Alberto Sulli and Serena Guiducci and Mislav Radić and Stefano Bombardieri and Martin Aringer and Franco Cozzi and Guido Valesini and Lidia Ananyeva and Gabriele Valentini and Gabriela Riemekasten and Maurizio Cutolo and Ruxandra Ionescu and L. Czirj{\'a}k and Nemanja Damjanov and Simona Rednic and {Matucci Cerinic}, Marco",
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AU - Cappelli, Susanna

AU - Bellando Randone, Silvia

AU - Martinović, Dušanka

AU - Tamas, Maria Magdalena

AU - Pasalić, Katarina

AU - Allanore, Yannick

AU - Mosca, Marta

AU - Talarico, Rosaria

AU - Opris, Daniela

AU - Kiss, C.

AU - Tausche, Anne Kathrin

AU - Cardarelli, Silvia

AU - Riccieri, Valeria

AU - Koneva, Olga

AU - Cuomo, Giovanna

AU - Becker, Mike Oliver

AU - Sulli, Alberto

AU - Guiducci, Serena

AU - Radić, Mislav

AU - Bombardieri, Stefano

AU - Aringer, Martin

AU - Cozzi, Franco

AU - Valesini, Guido

AU - Ananyeva, Lidia

AU - Valentini, Gabriele

AU - Riemekasten, Gabriela

AU - Cutolo, Maurizio

AU - Ionescu, Ruxandra

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KW - Rheumatoid arthritis

KW - Systemic lupus erythematosus

KW - Systemic sclerosis

KW - Undifferentiated connective tissue disease

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