MASSIVE PERICARDIAL EFFUSION IN SCLERODERMA: A REVIEW OF FIVE CASES

Medical records of five patients with sclerodenna (SSc), eachof whom had pericardial effusion with an estimated volume ofmore than 200 ml, were reviewed to study the clinical and immunologicalsignificance of massive pericardial effusion in SSc. DiffuseSSc (4/5), with a wide area of pigmentation (4/5), flexion contracture(4/5), oesophageal hypomotility (5/5), pulmonary fibrosis (4/5)and autoantibodies to topoisomerase I (3/5) were the commonfeatures in this group. High protein, lactate dehydrogenaseand low white blood cell count were the characteristics of pericardialfluid. None of the patients had signs of acute pericarditis.Four of the five cases died within 9 months of the diagnosisof pericarditis; two with renal failure, one with cardiac tamponadeand another with sudden death. The pericarditis in diffuse SSc,especially in cases with anti-topoisomerase I, may be characterizedby a chronic form of pericarditis with poor prognosis, oftencomplicated by ,renal failure. KEY WORDS: Scleroderma, Pericardial effusion, Antitopoisomerase I antibodies, Antinuclear antibodies, Renal failure