CASE-REPORT - 3 PEDIATRIC CASES OF PRIMARY SCLEROSING CHOLANGITIS TREATED WITH URSODEOXYCHOLIC ACID AND SULFASALAZINE

We present here three paediatric patients with primary sclerosing chol angitis. In case I, the serum gamma-glutamyl transpeptidase was decrea sed only temporarily by ursodeoxycholic acid (UDCA) treatment and 34 m onths later, sulphasalazine was added because of microscopic colitis. The enzyme level decreased with dual therapy. Similarly, in case 3, fi rst diagnosed as autoimmune hepatitis, the transpeptidase levels remai ned elevated for 18 months during treatment with UDCA, prednisolone an d mizoribin. The enzyme decreased only after a diagnosis of primary sc lerosing cholangitis complicated with ulcerative colitis was establish ed and sulphasalazine was introduced. Case 2 also had Crohn's colitis and was put on UDCA and sulphasalazine from the start. The enzyme leve l was normalized within 1 month and has remained normal for the follow ing 5 years. Liver biopsies were analysed repeatedly in these three pa tients. In case 1, periductal fibrosis remained unchanged while being treated by UDCA. There appeared to be no progression in liver cirrhosi s in case 3 while being treated by UDCA, prednisolone, and mizoribin. In case 2, who has been treated with both UDCA and sulphasalazine from the start, periductal fibrosis and portal fibrosis were remarkably im proved 45 months later. We suggest that sulphasalazine in addition to UDCA might be a viable treatment for children with primary sclerosing cholangitis.