Ulcerative colitis, autoimmune hemolytic anemia and primary sclerosing cholangitis in a child

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.

[1]  M. Corey,et al.  Primary sclerosing cholangitis in 32 children: Clinical, laboratory, and radiographic features, with survival analysis , 1995, Hepatology.

[2]  S. Hanauer,et al.  Cyclosporine in severe ulcerative colitis refractory to steroid therapy. , 1994, The New England journal of medicine.

[3]  R. Hultcrantz,et al.  Liver disease in ulcerative colitis: an epidemiological and follow up study in the county of Stockholm. , 1994, Gut.

[4]  D. Shouval,et al.  Sclerosing cholangitis associated with Crohn's disease and autoimmune haemolytic anaemia. , 1993, Postgraduate medical journal.

[5]  C. Elson,et al.  Red cell autoantibody production by colonic mononuclear cells from a patient with ulcerative colitis and autoimmune haemolytic anaemia , 1992, British journal of haematology.

[6]  D. Jewell,et al.  The association of autoimmune disorders with inflammatory bowel disease. , 1989, The Quarterly journal of medicine.

[7]  D. Vergani,et al.  Different immune mechanisms leading to autoimmunity in primary sclerosing cholangitis and autoimmune chronic active hepatitis of childhood , 1989, Hepatology.

[8]  D. Clements,et al.  Autoimmune haemolytic anaemia complicating ulcerative colitis. , 1988, British journal of hospital medicine.

[9]  B. Portmann,et al.  Primary sclerosing cholangitis in childhood. , 1987, Gastroenterology.

[10]  Moeller Dd.  Sclerosing cholangitis associated with autoimmune hemolytic anemia and hyperthyroidism. , 1985, American Journal of Gastroenterology.

[11]  M. Hadchouel,et al.  Sclerosing cholangitis in children. , 1994, The Journal of pediatrics.

[12]  R. Scully,et al.  Case records of the Massachusetts General Hospital. , 1990 .

[13]  G PLACITELLI,et al.  [Ulcerative colitis]. , 1958, La Riforma medica.