Mixed connective tissue disorder

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Template:WikiDoc Cardiology News Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Associate Editor: Cafer Zorkun, M.D., Ph.D. [2]

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.

• 
  Fibrous Pericarditis in MCTD
• 
  Cellular aggregates in perivascular space and adjacent myocardium

• 
  Cross Section of Intramural Coronary Artery
• 
  Small pulmonary artery in patient with MCTD and pulmnonary hypertension

Symptoms of MCTD

Common symptoms

• Raynaud’s phenomenon

• Swollen fingers or hands

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.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)

• 
  A teenager with MCTD presented as ACS before PCI
• 
  A teenager with MCTD presented as ACS post PCI

References

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