Scleroderma pericardial disease is usually silent and benign. The incidence
of pericardial involvement in scleroderma is about 50% according to autops
y results, but symptomatic pericarditis manifests in about 16% of patients
with diffuse scleroderma and in about 30% of patients with limited sclerode
rma. The clinically evident pericardial effusion is rare in scleroderma, al
though it can be detected in about 41% of patients with echocardiography. I
n majority of the patients, the pericardial effusion is small and not assoc
iated with symptoms. The pericardial effusion manifests usually after the m
anifestation of the other clinical and serologic features of scleroderma. A
case of scleroderma is reported that presented with a large pericardial ef
fusion, which antedated the other clinical and serologic features of sclero
derma. The pericardial involvement in scleroderma is reviewed.