Related Experiment Videos
Three paediatric cases of primary sclerosing cholangitis treated with ursodeoxycholic acid and sulphasalazine
K Kozaiwa1, H Tajiri, A Sawada
1Department of Paediatrics, Faculty of Medicine, Osaka University, Suita, Japan. kozaiwa@ped.med.osaka-u.ac.jp
Insights
Adding sulphasalazine to ursodeoxycholic acid (UDCA) treatment may improve outcomes for children with primary sclerosing cholangitis. This combination therapy showed positive results in liver enzyme levels and fibrosis in pediatric patients.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Autoimmune Diseases
Background:
- Primary sclerosing cholangitis (PSC) is a chronic liver disease affecting bile ducts.
- Treatment options for pediatric PSC are limited, often involving ursodeoxycholic acid (UDCA).
- Assessing the efficacy of combination therapies is crucial for improving patient outcomes.
Observation:
- Three pediatric patients with PSC were studied, with varying treatment approaches.
- Case 1 and 3 showed limited improvement with UDCA alone or in combination with other immunosuppressants.
- Case 2, treated with UDCA and sulphasalazine concurrently, achieved normalized liver enzymes and improved liver histology.
Findings:
- Combined therapy with UDCA and sulphasalazine demonstrated significant improvement in liver enzyme levels and reduced liver fibrosis in a pediatric PSC patient.
- Individual responses varied, suggesting a potential synergistic effect of sulphasalazine when added to UDCA.
- Histological analysis revealed remarkable improvement in periductal and portal fibrosis in the patient on dual therapy.
Implications:
- Sulphasalazine, in addition to UDCA, may represent a promising therapeutic strategy for pediatric primary sclerosing cholangitis.
- Further research is warranted to confirm the efficacy and safety of this combination therapy in a larger cohort.
- This finding could lead to improved management protocols for children suffering from PSC.
Abstract:
We present here three paediatric patients with primary sclerosing cholangitis. In case 1, the serum gamma-glutamyl transpeptidase was decreased only temporarily by ursodeoxycholic acid (UDCA) treatment and 34 months later, sulphasalazine was added because of microscopic colitis. The enzyme level decreased with dual therapy. Similarly, in case 3, first diagnosed as autoimmune hepatitis, the transpeptidase levels remained elevated for 18 months during treatment with UDCA, prednisolone and mizoribin. The enzyme decreased only after a diagnosis of primary sclerosing cholangitis complicated with ulcerative colitis was established and sulphasalazine was introduced. Case 2 also had Crohn's colitis and was put on UDCA and sulphasalazine from the start. The enzyme level was normalized within 1 month and has remained normal for the following 5 years. Liver biopsies were analysed repeatedly in these three patients. In case 1, periductal fibrosis remained unchanged while being treated by UDCA. There appeared to be no progression in liver cirrhosis 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 improved 45 months later. We suggest that sulphasalazine in addition to UDCA might be a viable treatment for children with primary sclerosing cholangitis.