Delayed response of IBD- associated autoimmune sclerosing cholangitis to standard immunosuppressive treatment in an 8-year old child

Autoimmune sclerosing cholangitis (ASC) is associated with inflammatory bowel disease in 45% of cases. Treatment consists of prednisolone 2 mg/kg/day tapered over 4-8 weeks to a maintenance dose of 2.5-5 mg/day, azathioprine (AZA) at 2.0-2.5 mg/kg/day is added in 85% of cases, and ursodeoxycholic acid (UDCA) at 15-20 mg/kg/day. In most patients an 80% reduction of transaminases occurs within the first two months of treatment. We present a case of an 8-year-old boy with pancolic ulcerative colitis and ASMA positive- ASC who presented a late response to immunosuppressive treatment. At presentation the patient’s blood tests showed: VES 120 mm/h, AST 360 U/L, ALT 329 U/L, FA 1314 U/L, FA/AST 3.65, ƳGT 499 U/L, total bilirubin 1.10 mg/dl, PT-INR 1.2, PTT ratio 1.62, albumin 3.3 g/dl, IgG 3582 mg/dl. After confirmation of ASC on cholangiogram and histology, prednisolone (2 mg/kg/day) was started and tapered over 8 weeks to a maintenance dose of 5 mg/day, with no modification of liver function tests (LFTs) except for normalization of PT and PTT. UDCA at 20 mg/kg/day was started since the beginning, AZA (2 mg/kg/day) was added at week 8 of steroid treatment prior to thiopurine methyltransferase genotype testing (no polymorphisms were identified). No reduction of transaminases and ƳGT was observed until month 6 of combined steroid and AZA treatment, complete normalization was observed at month 10. Currently the child is in remission with normal LFTs. The present describes the case of an ASMA positive –ASC with a late response to standard immunosuppressive treatment.