Mixed connective tissue disorder
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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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Fibrous Pericarditis in MCTD
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Cellular aggregates in perivascular space and adjacent myocardium
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Cross Section of Intramural Coronary Artery
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Small pulmonary artery in patient with MCTD and pulmnonary hypertension
Symptoms of MCTD
Common symptoms
- Swollen fingers or hands
Anti–U1 small nuclear RNP positive
Mixed Connective Tissue Disorder Findings
A.Systemic lupus erythematosus–like findings
- Facial erythema
- Leukopenia (<4,000/mm3) or thrombocytopenia (<100,000/mm3)
B.Progressive Systemic Sclerosis–like findings
- 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,
- Intimal hyperplasia of coronary arteries,
- Perivascular and myocardial leukocytic infiltrates,
- 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)[1]
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A teenager with MCTD presented as ACS before PCI
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A teenager with MCTD presented as ACS post PCI
References
- ↑ 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
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