What is the neonatal lupus syndrome?

The neonatal lupus syndrome is a disease of the fetus and neonate that likely results from maternal transfer of the autoantibodies anti-Ro (SS-A) and/or anti-La (SS-B) to the fetal circulation. Clinical manifestations are variable and include dermatitis, hepatitis, hemolytic anemia, and thrombocytopenia. The most common cardiac manifestation of the neonatal lupus syndrome is complete congenital heart block; this lesion is responsible for the major morbidity and mortality of this disorder. Many patients require permanent pacemaker therapy.

Polymyositis and dermatomyositis result in inflammation of skeletal muscle. Is cardiac muscle ever involved?

Both polymyositis and dermatomyositis are autoimmune disorders of unknown etiology; their primary clinical manifestation is weakness due to inflammation and necrosis of skeletal muscle. Helpful clinical findings include proximal muscle weakness, elevated serum enzymes (such as creatine phosphokinase), and characteristic electromyographic and biopsy abnormalities. Dermatomyositis is characterized by dermatologic findings in addition to myositis.

Up to 40% of patients with polymyositis and dermatomyositis may have cardiac abnormalities. Supraventricular tachyarrhythmias are most common. Various degrees of conduction defects and ventricular tachyarrhythmias also have been noted. An inflammatory myopathy of cardiac muscle resulting in congestive heart failure has occasionally been observed. Finally, pericarditis with varying degrees of effusion may occur. Coronary arteritis and valvular lesions are unusual.

What are the cardiac manifestations of scleroderma (systemic sclerosis)?

Systemic sclerosis is an autoimmune disorder of unknown etiology that results in a diffuse bland vasculopathy and variable fibrosis of skin and various end organs, particularly the heart, lungs, kidneys, and gastrointestinal tract. The diffuse vasculopathy may lead to varying degrees of ischemia and infarction in the skin and various end organs; the consequence of these diffuse ischemic events may be the characteristic fibrosis of the disease. Two major subcategories are often recognized. The first is systemic sclerosis with limited cutaneous scleroderma or CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia). The other is systemic sclerosis with diffuse cutaneous scleroderma.

The heart in systemic sclerosis may be involved directly, in the form of myocardial fibrosis, or indirectly, as a result of pulmonary or renal involvement. Clinical features include congestive heart failure, supraventricular and ventricular tachyarrhythmias, conduction disturbances, and syndromes of myocardial ischemia, including angina, acute myocardial infarction, and sudden death. Pericarditis also may be present, either as a primary manifestation or secondary to uremia.

What is the antiphospholipid antibody syndrome?

The antiphospholipid antibody syndrome (APS) is a disorder characterized by circulating antiphospholipid antibodies and manifesting clinically as variable degrees of recurrent arterial and venous thromboses, recurrent spontaneous abortions, and thrombocytopenia. The disease may be primary or associated with another disorder such as SLE. It remains unclear how the antiphospholipid antibodies result in clinical expression. Antiphospholipid antibodies may be recognized by the presence of the lupus anticoagulant (elevated partial thromboplastin time not corrected by addition of normal sera and not due to antibodies directed against specific clotting factors), a false-positive serologic test for syphilis, or the presence of anticardiolipin antibodies as determined by enzyme-linked immunosorbent assay.

Cardiac manifestations of APS include thromboses of the coronary arteries that lead to myocardial ischemia and/or infarction. Cardiac valvular abnormalities also may be present, including sterile vegetations and aortic and mitral regurgitation. Such lesions may occur in patients with primary APS as well as APS associated with SLE.

What is the neonatal lupus syndrome? Photo Gallery

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