Mixed connective tissue disease

Mixed connective tissue disease (MCTD), also known as Sharp's syndrome, is a rare systemic connective tissue disorder that characterized by a combination of some features of systemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, polymyositis with the presence of antibodies to s...

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Main Authors: R. U. Shayakhmetova, L. P. Ananyeva
Format: Article
Language:Russian
Published: IMA-PRESS LLC 2019-03-01
Series:Современная ревматология
Subjects:
Online Access:https://mrj.ima-press.net/mrj/article/view/880
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author R. U. Shayakhmetova
L. P. Ananyeva
author_facet R. U. Shayakhmetova
L. P. Ananyeva
author_sort R. U. Shayakhmetova
collection DOAJ
description Mixed connective tissue disease (MCTD), also known as Sharp's syndrome, is a rare systemic connective tissue disorder that characterized by a combination of some features of systemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, polymyositis with the presence of antibodies to soluble nuclear ribonucleoprotein (anti-U1-RNP) in high titers. The most common clinical manifestations of MCTD include Raynaud's phenomenon, hand edema, muscle weakness, arthralgia/arthritis, and esophageal hypotonia. The course of the disease is mostly benign; however, there are severe cases with damage to the lung, kidneys, cardiovascular system and central nervous system. Poor prognosis and the highest mortality rate are associated with pulmonary hypertension. The diagnosis of MCTD is difficult due to the absence of unified diagnostic criteria and lack of specific manifestations at the onset of the disease. Furthermore, there are no generally accepted guidelines for MCTD treatment.The paper considers the modern concepts of MCTD, its current diagnostic criteria, clinical and immunological features, and treatment.
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spelling doaj-art-48955def70f8453e97cd997a05b8c2f62025-08-20T03:59:56ZrusIMA-PRESS LLCСовременная ревматология1996-70122310-158X2019-03-01131111810.14412/1996-7012-2019-1-11-182141Mixed connective tissue diseaseR. U. Shayakhmetova0L. P. Ananyeva1V.A. Nasonova Research Institute of RheumatologyV.A. Nasonova Research Institute of RheumatologyMixed connective tissue disease (MCTD), also known as Sharp's syndrome, is a rare systemic connective tissue disorder that characterized by a combination of some features of systemic lupus erythematosus, systemic sclerosis, rheumatoid arthritis, polymyositis with the presence of antibodies to soluble nuclear ribonucleoprotein (anti-U1-RNP) in high titers. The most common clinical manifestations of MCTD include Raynaud's phenomenon, hand edema, muscle weakness, arthralgia/arthritis, and esophageal hypotonia. The course of the disease is mostly benign; however, there are severe cases with damage to the lung, kidneys, cardiovascular system and central nervous system. Poor prognosis and the highest mortality rate are associated with pulmonary hypertension. The diagnosis of MCTD is difficult due to the absence of unified diagnostic criteria and lack of specific manifestations at the onset of the disease. Furthermore, there are no generally accepted guidelines for MCTD treatment.The paper considers the modern concepts of MCTD, its current diagnostic criteria, clinical and immunological features, and treatment.https://mrj.ima-press.net/mrj/article/view/880mixed connective tissue diseaseanti-u1-rnpoverlap syndrome.
spellingShingle R. U. Shayakhmetova
L. P. Ananyeva
Mixed connective tissue disease
Современная ревматология
mixed connective tissue disease
anti-u1-rnp
overlap syndrome.
title Mixed connective tissue disease
title_full Mixed connective tissue disease
title_fullStr Mixed connective tissue disease
title_full_unstemmed Mixed connective tissue disease
title_short Mixed connective tissue disease
title_sort mixed connective tissue disease
topic mixed connective tissue disease
anti-u1-rnp
overlap syndrome.
url https://mrj.ima-press.net/mrj/article/view/880
work_keys_str_mv AT rushayakhmetova mixedconnectivetissuedisease
AT lpananyeva mixedconnectivetissuedisease