Acute congestive heart failure associated with a limited form of systemic sclerosis and primary biliary cirrhosis.

This is the first case of a limited form of systemic sclerosis (ISSc) associated with acute congestive heart failure (CHF) and primary biliary cirrhosis (PBC). A 58-year-old woman with ISSc was admitted because of a sudden onset of CHF. The intravenous administration of nitroglycerine and furosemide ameliorated the symptoms of CHF within 24 hours. She had both anticentromere antibodies and anti-p25 doublet/triplet antibodies to intrahepatic microsomes. Thallium scintigraphy at rest demonstrated significant perfusion defects in both the anteroseptal and inferior myocardium. A coronary angiogram revealed normal coronary arteries and no vasospasm was provoked by the intracoronary administration of acetylcholine. The present case indicates that minute care should thus be taken for the prevention of acute CHF even in patients with a limited form of SSc when thallium perfusion defects are identified.