[Primary sclerosing cholangitis in isolated IgA deficiency].

Primary sclerosing cholangitis was diagnosed in a patient with isolated IgA deficiency. Similar reports in the literature suggest that the two conditions are related. Patients with primary sclerosing cholangitis associated with isolated IgA deficiency are distinguished by the fact that it is mainly the intrahepatic bile ducts that are narrowed by the fibrosing process. The disease manifests itself comparatively early. Recurrent bouts of fever, in combination with elevated AP, can become the leading symptom even before the onset of jaundice. The prognosis seems somewhat more favourable than in other patients with primary sclerosing cholangitis.