Diffuse Pancreatic Fibrosis: An Uncommon Feature of Multifocal Idiopathic Fibrosclerosis

Abstract

Multifocal idiopathic fibrosclerosis (MIF) is a rare syndrome characterized by exuberant fibrosis involving diverse organ systems. MIF is manifest by varying combinations of the following conditions: mediastinal fibrosis, retroperitoneal fibrosis, orbital pseudotumor, Riedel's thyroiditis, and sclerosing cholangitis. Less common features of MIF include Dupuytren's contractures, lymphoid hyperplasia, Peyronie's disease, vasculitis, testicular fibrosis, and pachymeningitis. Fibrosis arising from the pancreas has been previously described in two patients with MIF. We report a 58-yr-old white man with MIF manifest as orbital pseudotumor, sclerosing cholangitis, lymph node hyperplasia, and diffuse pancreatic fibrosis.