Pericardial disease in scleroderma (systemic sclerosis)

Abstract

A review of the records of 210 patients with scleroderma seen between 1952 and 1972 revealed two clinical patterns of pericardial disease in 15 patients: (1) Chronic pericardial effusion (11 patients), confirmed by roentgenography and ultrasound, occurred in association with dyspnea, Raynaud's syndrome, cardiomegaly, congestive heart failure and pleural effusion in the absence of renal failure. In three patients hemodynamic signs of pulsus paradoxus, Kussmaul's sign or pulsus alternans developed. In six patients with chronic effusion renal failure developed within 6 months, an incidence severalfold higher than expected in the scleroderma population at large. (2) Acute pericarditis (four patients) was associated with dyspnea, chest pain, pericardial friction rub, fever, cardiomegaly and elevated latex fixation titers (in two of four patients). Pericardial disease is a recognizable clinical entity in scleroderma and should be considered in all patients with cardiomegaly, congestive heart failure or chest pain. In 34 autopsy studies, the incidence of pericardial involvement (62 per cent) exceeded the incidence of significant myocardial fibrosis (30 per cent); thus pericardial scleroderma represents a relatively common form of cardiac involvement in this diffuse connective tissue disease.