Idiopathic retroperitoneal fibrosis and mediastinal fibrosis are localized expressions of a systemic sclerosing disease of unknown cause which, on rare occasions, may coexist in the same patient. Although pericardial involvement may occur, recurrent constrictive pericarditis that is unrelieved by pericardiectomy has not previously been reported in association with either idiopathic retroperitoneal or mediastinal fibrosis. Reported herein is a case of recurrent constrictive pericarditis that was unrelieved by two pericardiectomies, and autopsy revealed unsuspected combined idiopathic retroperitoneal and mediastinal fibrosis. The clinical, echocardiographic, and pathologic findings are described.
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