Mixed connective tissue disease overview: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
MCTD is a [[Systemic autoimmune diseases|systemic autoimmune disease]] that characterized by overlapping features between two or more [[systemic autoimmune diseases]] ([[Systemic lupus erythematosus|systemic lupus erythematosus (SLE)]], [[Rheumatoid arthritis|rheumatoid arthritis (RA)]], [[Dermatomyositis|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]], [[Immunology|immunological]] and [[Inflammation|inflammatory]] processes and excessive [[fibrosis]] caused by redundant synthesis of [[collagen]] and other [[Matrix protein|matrix proteins]]. In MCTD associated conditions include secondary [[Sjögren's syndrome|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 [[Histopathology|histopathological]] abnormalities of [[Skin lesion|skin lesions]] include poor and [[Lichen|lichenoid]] interface [[dermatitis]] and suprabasilar [[exocytosis]] around [[Necrosis|necrotic]] [[Keratinocyte|keratinocytes]]. | |||
==Pathophysiology== | |||
==Causes== | ==Causes== | ||
Revision as of 19:27, 17 April 2018
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Mixed connective tissue disease Microchapters |
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Differentiating Mixed connective tissue disease from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Mixed connective tissue disease overview On the Web |
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Risk calculators and risk factors for Mixed connective tissue disease overview |
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.