The heart in progressive systemic sclerosis (scleroderma)
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Overview
Cardiac disease is one of the major causes of death in Progressive Systemic Sclerosis (Scleroderma) and it is plausible that cardiac disease contributes to mortality from other causes in SSc [1]. Subclinical cardiac involvement, including conduction abnormalities, myocardial and pericardial disease, is thought to be common in scleroderma.[1] Autopsy studies have suggested a high frequency of abnormalities, particularly replacement fibrosis and contraction band necrosis [1].
All cardiac structures may be involved, resulting in pericardial effusion, atrial and ventricular arrhythmias, conduction system defects, valvular abnormalities, myocardial ischaemia, myocardial hypertrophy and heart failure. ‘Primary’ myocardial involvement, without systemic or pulmonary hypertension and without significant renal or pulmonary involvement, may be the consequence of the characteristic vascular lesions of this disease. The prevalence of cardiac disease varies depending on its definition. Clinical symptoms or abnormalities on ECG, chest X-ray or echocardiography have been proposed as candidate variables for routine cardiac assessment.[1]
Clinically apparent cardiac disease has been reported in only 10% of the patients and has been associated with an adverse prognosis. In addition to fibrotic changes in the myocardium, a ‘cardiac Raynaud’s phenomenon’ involving small myocardial vessels and an immune-mediated myocarditis has been suggested, but the exact pathogenesis of myocardial involvement in scleroderma is unclear.
Although cardiac abnormalities may be more prevalent and severe in the diffuse cutaneous subtype of the disease (which has been the most intensively investigated), there is increasing evidence suggesting that cardiac involvement is also a frequent finding in the limited cutaneous subtype. In the large epidemiological Italian study, although heart symptoms were found more frequently in the diffuse subtype (32%) as compared with the limited form (23%), the difference was not statistically significant.[1]
The results of an angiographic based study among 172 patients suggests that the prevalence of obstructive epicardial disease in scleroderma is probably similar to that of individuals without scleroderma. Therefore, scleroderma patients with suspected CAD should be managed in the same way as patients without scleroderma[1].
References
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
