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Mixed connective tissue disease — enigma variations?

Ciang, NCO; Pereira, N; Isenberg, DA; (2017) Mixed connective tissue disease — enigma variations? Rheumatology , 56 (3) pp. 326-333. 10.1093/rheumatology/kew265. Green open access

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Abstract

In 1972, Sharp and colleagues described a new autoimmune rheumatic disease which they called mixed connective tissue disease (MCTD), characterized by overlapping features of systemic sclerosis (SSc), systemic lupus erythematosus (SLE), polymyositis/dermatomyositis (PM/DM), high levels of anti-U1snRNP and low steroid requirement use with good prognosis. MCTD was proposed as a distinct disease. However, soon after the original description, questions about the existence of such a syndrome as well as disputes over the features initially described began to surface. The conundrum of whether MCTD is a distinct disease entity remains controversial. We undertook a literature review focusing on the articles reporting new data about MCTD published in the last decade, to determine whether any new observations help to answer the conundrum of MCTD. After reviewing recent data, we question whether the term MCTD is appropriately retained, preferring to use the term “undifferentiated autoimmune rheumatic disease”.

Type: Article
Title: Mixed connective tissue disease — enigma variations?
Open access status: An open access version is available from UCL Discovery
DOI: 10.1093/rheumatology/kew265
Publisher version: http://dx.doi.org/10.1093/rheumatology/kew265
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: mixed connective tissue disease, anti-U1snRNP, clinical features, pulmonary hypertension, capillaroscopy, immunology, epitope spreading, antigen modification
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/1502169
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