A 70-year-old man who had been diagnosed with retroperitoneal fibrosis (RPF) was admitted to our hospital complaining of dyspnea. Imaging studies showed massive pericardial effusion. His condition deteriorated and pericardiostomy was performed. A biopsy of the pericardium revealed marked fibrosis with infiltration of lymphocytes, which was identical to RPF findings. A diagnosis of multifocal fibrosclerosis was made. Despite aggressive treatment, he died with clinical signs of cardiovascular failure. The autopsy specimen revealed proliferation of fibrosis with infiltration of lymphocytes in multiple organs. Even after successful decompression of urinary obstruction for RPF, long-term follow-up is necessary in these patients because of the possibility of other fatal complications such as pericardial fibrosis.
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