MASSIVE PERICARDIAL-EFFUSION IN SCLERODERMA - A REVIEW OF 5 CASES

Medical records of five patients with scleroderma (SSc), each of whom had pericardial effusion with an estimated volume of more than 200 ml, were reviewed to study the clinical and immunological significance of massive pericardial effusion in SSc. Diffuse SSc (4/5), with a wide a rea of pigmentation (4/5), flexion contracture (4/5), oesophageal hypo motility (5/5), pulmonary fibrosis (4/5) and autoantibodies to topoiso merase I (3/5) were the common features in this group. High protein, l actate dehydrogenase and low white blood cell count were the character istics of pericardial fluid. None of the patients had signs of acute p ericarditis. Four of the five cases died within 9 months of the diagno sis of pericarditis; two with renal failure, one with cardiac tamponad e and another with sudden death. The pericarditis in diffuse SSc, espe cially in cases with anti-topoisomerase I, may be characterized by a c hronic form of pericarditis with poor prognosis, often complicated by renal failure.