Sarcoidosis-induced pericarditis in a patient with portopulmonary hypertension: a case report

Abstract

Portopulmonary hypertension is a rare and severe complication of patients with cirrhosis. Sarcoidosis, a disease of unknown etiology, is also a cause of pulonary hypertension and right heart dysfunction. We report the case of a 51-year-old male patient, suffering from cirrhosis due to Wilson’s disease, portal hypertension and pulmonary hypertension (PH), who developed severe pericarditis. Wilson’s disease was diagnosed 8 years before his last admission to our hospital and was being successfully treated with D-penicillamine. PH was recognized 2 years before admission and being treated with bosentan. The patient complained for dyspnea at rest and the 2D echocardiogram revealed a significant amount of pericardial fluid. All other causes of acute pericarditis were excluded and his laboratory, imaging and histopathological investigation showed evidence of sarcoidosis. He underwent a therapy with corticosteroids (methylprednisolone) and his follow-up examination showed remarkable decrease of the levels of mean pulmonary artery pressure and pericardial fluid.

A Case Report of a Patient with Wilson’s Disease and Coincidental Sarcoidosis

“…To our knowledge, this is the third case of coexistence of Wilson's disease and sarcoidosis that has been reported [Danks DM et al, 1990], [Giouleme O. et al, 2009] It was thought that portopulmonary hypertension was a vascular consequence of the advanced liver disease caused by Wilson's disease. The authors' further investigation of the acute pericarditis provided evidence of sarcoidosis, as another factor of secondary pulmonary arterial hypertension.…”