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| __NOTOC__
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| {{Mixed connective tissue disease}}
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| {{SI}}
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| {{CMG}}; {{AE}} {{CZ}}, {{JH}}, {{MIR}}
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| {{SK}} MCTD; mixed connective tissue disorder; Sharp's syndrome
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| ==Overview==
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| '''Mixed connective tissue disease''' ('''MCTD''') or ''Sharp's syndrome'' is a human [[autoimmune disease]] in which the [[immune system]] attacks the body. MCTD combines features of [[polymyositis]], [[systemic lupus erythematosus]], and [[systemic scleroderma]] and is thus considered an [[overlap syndrome]]. MCTD commonly causes [[arthritis|joint pain/swelling]], [[Raynaud phenomenon]], [[myositis|muscle inflammation]], and [[sclerodactyly|scarring of the skin of the hand]]. It does not typically cause [[kidney]] disease or [[seizure]]s. Distinguishing laboratory characteristics are a positive, speckled [[anti-nuclear antibody]] and an [[anti-U1-RNP antibody]].<ref>Venables PJ. ''Mixed connective tissue disease.'' Lupus. 2006;15(3):132-7. PMID 1663436</ref>
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| ==Pathophysiology==
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| There are no specific histologic findings that aid in the diagnosis of [[Mixed Connective Tissue Disorder]] as a separate autoimmune disease. For example, [[nephritis]] in [[MCTD]] is usually indistinguishable from [[lupus]] nephritis.
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| <div align="left">
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| <gallery heights="225" widths="225">
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| image:Fibrous Pericarditis in MCTD.jpg|Fibrous Pericarditis in MCTD
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| image:Cellular aggregates in perivascular space and adjacent myocardium.jpg|Cellular aggregates in perivascular space and adjacent myocardium
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| image:Cross Section of Intramural Coronary Artery.jpg|Cross Section of Intramural Coronary Artery
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| image:Small pulmonary artery in patient with MCTD and pulmonary hypertension.jpg|Small pulmonary artery in patient with MCTD and pulmnonary hypertension
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| </gallery>
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| </div>
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| ==Historical Perspective==
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| ==Classification==
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| ==Pathophysiology==
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| *Associated Conditions:
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| **[[Systemic lupus erythematosus]]–like findings
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| ***[[Polyarthritis]]
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| ***[[Lymphadenopathy]]
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| ***Facial [[erythema]]
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| ***[[Pericarditis]] or [[pleuritis]]
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| ***[[Leukopenia]] (<4,000/mm3) or thrombocytopenia (<100,000/mm3)
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| **[[Scleroderma|Progressive Systemic Sclerosis]] –like findings
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| ***[[Sclerodactyly]]
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| ***[[Pulmonary fibrosis]], restrictive changes of the lung (forced vital capacity <80% of predicted), or reduced carbon monoxide diffusing capacity (<70% of predicted)
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| **[[Polymyositis]]-like findings
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| ==Causes==
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| ==Differentiating Mixed Connective Tissue Disease from other Diseases==
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| ==Epidemiology and Demographics==
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| ===Age===
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| ===Gender===
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| ===Race===
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| ==Risk Factors==
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| ==Natural History, Complications and Prognosis==
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| Cardiovascular abnormalities associated with mixed connective tissue disease are rare. Presence of any of the complications listed below indicates unfavorable prognosis;
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| *Acute [[pericarditis]] and/or [[pericardial effusion]],
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| *[[Mitral valve prolapse]],
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| *Intimal hyperplasia of coronary arteries,
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| *Perivascular and myocardial leukocytic infiltrates,
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| *[[Pulmonary hypertension]].
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| *Coronary Artery Disease (although it is rarer, ACS may occur secondary to vascular changes and underlying CAD. The youngest patient with MCTD and ACS in literature was 18 years old)<ref>Jang J J, Olin J W, Fuster V. A teenager with mixed connective tissue disease presenting with an acute coronary syndrome. Vascular Medicine; 2004, Vol. 9 Issue 1, 31-34</ref>
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| <div align="left">
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| <gallery heights="225" widths="225">
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| image:Teenager before PCI.jpg|A teenager with MCTD presented as ACS before PCI
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| image:Teenager post PCI.jpg|A teenager with MCTD presented as ACS post PCI
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| </gallery>
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| </div>
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| ==Diagnosis==
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| ===Diagnostic Criteria===
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| ===Symptoms===
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| *[[Raynaud’s phenomenon]]
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| *Swollen fingers or hands
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| *Muscle weakness
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| ===Physical examination===
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| ====Gallery====
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| =====Trunk=====
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| <gallery>
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| Image: Mixed_connective_tissue_disease_01.jpg|Mixed connective tissue disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=294>
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| </gallery>
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| =====Extremities=====
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| <gallery>
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| Image: Mixed_connective_tissue_disease_02.jpg|Mixed connective tissue disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=294>
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| Image: Mixed_connective_tissue_disease_03.jpg|Mixed connective tissue disease. <SMALL><SMALL>''[http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=12 With permission from Dermatology Atlas.]''<ref name="www.atlasdermatologico.com.br">{{Cite web | title = Dermatology Atlas | url = http://www.atlasdermatologico.com.br/disease.jsf?diseaseId=294>
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| </gallery>
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| ===Laboratory Findings===
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| *Elevated serum level of muscle enzymes (creatine kinase)
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| *MCTD is characterized by anti–U1 small nuclear RNP positivity.
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| ===Imaging Findings===
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| **Myogenic pattern on electromyogram
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| ===Other Diagnostic Studies===
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| **Hypomotility or dilatation of esophagus
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| == References ==
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| {{reflist|2}}
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