Mixed connective tissue disease overview: Difference between revisions
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===MRI=== | ===MRI=== | ||
[[Musculoskeletal system|Musculoskeletal]] [[Magnetic resonance imaging|MRI]] can identify and characterize subclinical [[Synovitis|synovial inflammation]] and [[joint]] damage with a greater [[precision]] than [[X-rays]]. Also cardiac [[Magnetic resonance imaging|MRI]] is complementary for diagnosing [[Pulmonary hypertension|pulmonary arterial hypertension]]. | |||
===Other Imaging Findings=== | ===Other Imaging Findings=== |
Revision as of 20:57, 17 April 2018
Mixed connective tissue disease Microchapters |
Differentiating Mixed connective tissue disease from other Diseases |
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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.
Differentiating mixed connective tissue disease from Other Diseases
Cystic fibrosis has to be differentiated from other conditions with similar presentation of arthritis and rash like systemic lupus erythematosus, rheumatoid arthritis, rhupus, undifferentiated connective tissue disease, systemic sclerosis, Sjogren's syndrome, vasculitis, Behcet's syndrome, Kikuchi's disease, serum sickness, psoriatic arthritis and human parvovirus B19 infection.
Epidemiology and Demographics
Risk Factors
There are no established risk factors for mixed connective tissue diease.
Screening
There is insufficient evidence to recommend routine screening for mixed connective tissue disease.
Natural History, Complications, and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Musculoskeletal MRI can identify and characterize subclinical synovial inflammation and joint damage with a greater precision than X-rays. Also cardiac MRI is complementary for diagnosing pulmonary arterial hypertension.