The heart in progressive systemic sclerosis (scleroderma)

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2] Assistant Editor-In-Chief: Brian Blank

Severe complications from Progressive Systemic Scleroderma (Scleroderma) can include:

• Untreated high blood pressure that strains the heart
• enlargement of the heart, which leads to heart failure

• pericardial effusion

• arrhythmias (both atrial and ventricular)

• conduction system defects

• valvular abnormalities

• myocardial ischaemia

• myocardial hypertrophy^[1]

It’s generally thought cardiac disease is a major cause of death from scleroderma, or that it often contributes to death, though cardiac disease is only reported in about 10 percent of cases. Conduction abnormalities, myocardial and pericardial disease disease may also be common. Some have suggested a ‘cardiac Raynaud’s phenomenon‘ exists, but exactly how myocardial involvement develops in scleroderma is unclear.

An angiography-based study of 172 patients has suggested patients who may have obstructive epicardial disease associated with scleroderma should be treated the same as patients without scleroderma.^[1]

Images courtesy of Professor Peter Anderson DVM PhD and published with permission © PEIR, University of Alabama at Birmingham, Department of Pathology

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