Mixed connective tissue disease overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shaghayegh Habibi, M.D.[2]
Overview
Historical Perspective
MCTD was first defined by Gordon C.Sharp et al., in 1972. It has been the first rheumatic disease syndrome defined by a serologic test. In 1976, Alarcon-Segovia proposed criteria for classifying MCTD among all types of connective tissue diseases. It demonstrates the close association between MCTD and Sjogren's syndrome.
Classification
There is no established system for the classification of mixed connective tissue disease.
Pathophysiology
MCTD is a systemic autoimmune disease that characterized by overlapping features between two or more systemic autoimmune diseases (systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), dermatomyositis (DM), polymyositis, and scleroderma) and the presence of antibodies against U1snRNP. Main pathogenetic mechanisms in mixed connective tissue disease include vasculopathy which leads to tissue ischemia, immunological and inflammatory processes and excessive fibrosis caused by redundant synthesis of collagen and other matrix proteins. In MCTD associated conditions include secondary Sjogren’s syndrome and trigeminal neuralgia. A significant association of U1RNP disease with HLA-DR4 and DR154-61 is noted. Gross pathology of skin may include photo-distributed erythematosus annular lesions and papulosquamous lesions and the histopathological abnormalities of skin lesions include poor and lichenoid interface dermatitis and suprabasilar exocytosis around necrotic keratinocytes.
Causes
Mixed connective tissue disease is an autoimmune disease and the exact cause is unknown.