Abstract

A 15-month-old female who initially presented with autoimmune hemolytic anemia (AIHA) is described. She developed bloody stools and was diagnosed with ulcerative colitis (UC). Investigations of persistent hepatomegaly revealed primary sclerosing cholangitis (PSC). The association of AIHA, UC and PSC has never been reported. All these conditions entail impaired immunoregulation. Patients with a clustering of autoimmune diseases may help to delineate the pathogenesis of UC. Autoimmune phenomena may be prominent in inflammatory bowel disease. UC, in particular, exhibits a high incidence of associated autoimmune diseases including hypothyroidism, PSC, vitiligo and alopecia areata. AIHA is well described in 0.5% to 1.0% of adult UC patients but has not been reported in children with UC.