Am J Gastroenterol 1987 Nov;82(11):1196-202

Sclerosing cholangitis associated with chronic cryptosporidiosis in a child wit
a congenital immunodeficiency disorder.

Davis JJ, Heyman MB, Ferrell L, Kerner J, Kerlan R Jr, Thaler MM

Department of Pediatrics, University of California, San Francisco.

Abstract
Primary sclerosing cholangitis (PSC) in children is a rare and incurable
disorder of unknown etiology. We report an immunodeficient child with chronic
cryptosporidiosis of the biliary tract leading to clinical, pathological, and
radiographic findings consistent with PSC. This case documents the ability of
Cryptosporidium to disseminate to extraintestinal organs, and suggests that
chronic cryptosporidial infection of the biliary tract may be one etiological
mechanism producing sclerosing cholangitis in immunodeficient children. The
increased incidence of PSC in immunodeficient children may in part be due to
their inability to resolve infections of the biliary tract, which may result in
sclerosing cholangitis mimicking PSC. We submit that an aggressive diagnostic
workup should be performed to rule out an infectious etiology of sclerosing
cholangitis in immunodeficient patients who have findings of PSC, because
specific chemotherapy against the infecting organism would potentially arrest
progressive biliary obliteration