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Vol. 150. Issue 1.
Pages 26-32 (January 2018)
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Vol. 150. Issue 1.
Pages 26-32 (January 2018)
Review
Facts and controversies in mixed connective tissue disease
Hechos y controversias en la enfermedad mixta del tejido conectivo
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Julia Martínez-Barrioa,b,c,
Corresponding author
juliamartinezbarrio@gmail.com

Corresponding author.
, Lara Valora,b, F. Javier López-Longoa,b,c
a Servicio de Reumatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain
b Instituto de Investigación Biomédica Hospital Gregorio Marañón, Madrid, Spain
c Universidad Complutense de Madrid, Madrid, Spain
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Tables (4)
Table 1. Classification criteria for the mixed connective tissue disease proposed by Alarcon-Segovia and Villarreal.
Table 2. Classification criteria for mixed connective tissue disease proposed by Sharp.
Table 3. Classification criteria for mixed connective tissue disease proposed by Kasukawa et al.
Table 4. Proposed activity criteria for mixed connective tissue disease.
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Abstract

Mixed connective tissue disease (MCTD) is a systemic autoimmune rheumatic disease (SARD) characterized by the combination of clinical manifestations of systemic lupus erythematosus (SLE), cutaneous systemic sclerosis (SSc) and polymyositis-dermatomyositis, in the presence of elevated titres of anti-U1-RNP antibodies. Main symptoms of the disease are polyarthritis, hand oedema, Raynaud's phenomenon, sclerodactyly, myositis and oesophageal hypomobility.

Although widely discussed, most authors today accept MCTD as an independent entity. Others, however, suggest that these patients may belong to subgroups or early stages of certain definite connective diseases, such as SLE or SSc, or are, in fact, SARD overlap syndromes.

Keywords:
Mixed connective tissue disease
Seronegative
Anti-U1-RNP
Resumen

La enfermedad mixta del tejido conectivo (EMTC) es una enfermedad reumática autoinmunitaria sistémica (ERAS) caracterizada por la asociación de manifestaciones clínicas de lupus eritematoso sistémico (LES), esclerosis sistémica cutánea (ESC) y polimiositis-dermatomiositis en presencia de títulos elevados de anticuerpos anti-U1-RNP en el suero de los pacientes. Sus principales síntomas son la poliartritis, el edema de manos, el fenómeno de Raynaud, la esclerodactilia, la miositis y la hipomotilidad esofágica.

Actualmente, la mayoría de los autores acepta que la EMTC es una entidad independiente, pero algunos mantienen que estos pacientes podrían presentar una ERAS, definida en su fase precoz como LES o ESC, o ser, en realidad, un síndrome de solapamiento de la ERAS.

Palabras clave:
Enfermedad mixta del tejido conectivo
Seronegativo
Anti-U1-RNP

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