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

Autoimmune sclerosing cholangitis (ASC) is frequently associated with inflammatory bowel disease (IBD) and in most cases liver disease responds to the same immunosuppressant therapy for IBD, within the first few months. We describe the case of an 8-year-old boy with pancolic ulcerative colitis (UC) and ASMA positive ASC, whose liver disease had a peculiar response to standard treatment. At onset our patient presented with bloody diahrroea, increased inflammatory markers (VES 120 mm/h), hypertransaminasemia (AST 360 UI/L, ALT 329 UI/L), cholestasis (FA 1314 U/L, γGT 499 U/L, FA/AST 3.65), hypergammaglobulinemia (IgG 3582 mg/dl) and positive ASMA antibodies. After histological confirmation of UC and ASC, prednisolone (2 mg/kg/day) was started and tapered over 8 weeks to a maintenance dose of 5 mg/day, with regression of bloody diarrhoea but no modification of liver tests (LTs). UDCA at 20 mg/kg/day was started since the beginning, AZA (2 mg/kg/day) was added at week 8 of steroid treatment, after thyopurine methyltransfersae genotyping did not reveal polymorphisms. During the first 6 months of combined steroid and AZA treatment, intestinal symptoms remitted but there was no improvement of LTs; reduction was seen starting from month 8 and complete normalization at month 10, currently maintained at 16 months after diagnosis. In literature, definition and management of refractory ASC is scarse. Our patient's liver disease presented a prolonged period of refractoriness to standard immunosuppressive treatment, despite intestinal remission. Response was delayed but occurred without requiring escalation to an alternative treatment.