SCLEROSING CHOLANGITIS IN CHILDHOOD: AN 18-YEAR EXPERIENCE

Abstract

160 Sclerosing cholangitis (SC) in childhood is a heterogeneous condition. To define the relative incidence and prognosis of the different forms of SC, we have reviewed the features and outcome of 58 children diagnosed in our Unit between 1978-96. 37 (68%) [median age: 11 (2-16) yrs] had SC with serological features of autoimmunity [autoimmune SC (ASC)]; 5 (9%) [median age: 0.25(0.25-4) yrs] had neonatal SC (NSC); 6 (10%) [median age: 5 (3-14) yrs] had SC with other disorders [ulcerative colitis (UC) in 3, thyroiditis in 1, Langerhan cell histiocytosis (LCH) in 2]; 5 (9%) [median age: 7 (4-15) yrs] had SC associated with immunodeficiency (ID); 5 (9%) [median age: 5 (2-8) yrs] had no detectable association [primary SC (PSC)]. 29 (50%) were boys. Cholangiography showed abnormalities of intrahepatic ducts in 26 (45%), intra- and extrahepatic ducts in 32 (55%). At diagnosis, 30 (55%) were jaundiced (23 ASC, 5 NSC, 2 SC with LCH), 32 (55%) (16 ASC, 5 SC with ID, 5 SC with other disorders, 3 PSC, 3 NSC) had hepatosplenomegaly, 22 (38%) had normal serum alkaline phosphatase levels (18 ASC, 1 SC with ID, 2 SC with other disorders, 1 PSC), 14 (10 ASC, 2 SC with other disorders, 1 SC with ID, 1 PSC) had normalγ glutamyl transpeptidase. ANCA were positive in 29 (50%). On initial liver biopsy, biliary cirrhosis was noted in 13 (22%) (6 ASC, 1 SC with other disorders, 2 SC with ID, 2 PSC, 2 NSC). 35 (33 ASC, 2 SC with UC) were treated with immunosuppression, 31 (12 ASC, 5 SC with ID, 5 SC with other disorders, 4 PSC, 5 NSC) with ursodeoxycholic acid. Median follow up is 4 (1-18) yrs. 43(74%) are alive, 5 lost to follow up, 10 (17%) (3 ASC, 2 SC with ID, 2 SC with LCH, 1 PSC, 2 NSC) have died and/or had liver transplant from 1 to 9 (median: 2) years after diagnosis. Our results show that the most common form of SC in childhood is ASC. PSC, ie, SC without recognisable association, is rare.