Mixed connective tissue disorder: Difference between revisions

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#REDIRECT:[[Mixed connective tissue disease]]
 
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==Histopathological Findings==
 
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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<gallery heights="225" widths="225">
image:Fibrous Pericarditis in MCTD.jpg|Fibrous Pericarditis in MCTD
image:Cellular aggregates in perivascular space and adjacent myocardium.jpg|Cellular aggregates in perivascular space and adjacent myocardium
</gallery>
</div>
 
 
<div align="left">
<gallery heights="225" widths="225">
image:Cross Section of Intramural Coronary Artery.jpg|Cross Section of Intramural Coronary Artery
image:Small pulmonary artery in patient with MCTD and pulmonary hypertension.jpg|Small pulmonary artery in patient with MCTD and pulmnonary hypertension
</gallery>
</div>
 
==Symptoms of MCTD==
 
===Common symptoms===
 
*[[Raynaud’s phenomenon]]
 
*Swollen fingers or hands
 
===Anti–U1 small nuclear RNP positive===
 
===Mixed Connective Tissue Disorder Findings===
 
A.[[Systemic lupus erythematosus]]–like findings
 
*[[Polyarthritis]]
 
*[[Lymphadenopathy]]
 
*Facial [[erythema]]
 
*[[Pericarditis]] or [[pleuritis]]
 
*[[Leukopenia]] (<4,000/mm3) or thrombocytopenia (<100,000/mm3)
 
B.[[Scleroderma|Progressive Systemic Sclerosis]]–like findings
 
*[[Sclerodactyly]]
 
*[[Pulmonary fibrosis]], restrictive changes of the lung (forced vital capacity <80% of predicted), or reduced carbon monoxide diffusing capacity (<70% of predicted)
 
*Hypomotility or dilatation of esophagus
 
C.[[Polymyositis]]-like findings
 
*Muscle weakness
 
*Elevated serum level of muscle enzymes (creatine kinase)
 
*Myogenic pattern on electromyogram
 
==Cardiac Involvement in MCTD==
 
Cardiovascular abnormalities associated with mixed connective tissue disease are rare. Presence of any of the complications listed below indicates unfavorable prognosis;
 
*Acute [[pericarditis]] and/or [[pericardial effusion]],
 
*[[Mitral valve prolapse]],
 
*Intimal hyperplasia of coronary arteries,
 
*Perivascular and myocardial leukocytic infiltrates,
 
*[[Pulmonary hypertension]].
 
*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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<gallery heights="225" widths="225">
image:Teenager before PCI.jpg|A teenager with MCTD presented as ACS before PCI
image:Teenager post PCI.jpg|A teenager with MCTD presented as ACS post PCI
</gallery>
</div>
 
==References==
{{reflist|2}}
 
 
 
[[Category:Cardiology]]
[[Category:Rheumatology]]
 
[[fr:Syndrome de Sharp]]
[[nl:Mixed connective tissue disease]]
[[ja:混合性結合組織病]]
[[sv:Mixed Connective Tissue Disease]]
 
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Latest revision as of 18:07, 2 November 2012