Combined Therapy with Azathioprine, Prednisolone, and Ursodiol in Patients with Primary Sclerosing Cholangitis: A Case Series

21 December 1999 | Volume 131 Issue 12 | Pages 943-946

Background: No established medical therapy alters the progressive course of primary sclerosing cholangitis.

Objective: To explore the potential usefulness of combined therapy with azathioprine, steroids and ursodeoxycholic acid (UDCA) in primary sclerosing cholangitis.

Design: Case series.

Setting: University hospital in Mainz, Germany.

Patients: 15 patients with primary sclerosing cholangitis.

Intervention: Azathioprine (1 to 1.5 mg/kg of body weight per day), prednisolone (1 mg/kg per day initially, tapering to 5 to 10 mg per day) and UDCA (500 to 750 mg per day).

Measurements: Clinical and laboratory evaluation, liver biopsy, and endoscopic retrograde cholangiography (a >30% change in stenosis was considered significant).

Results: After a median observation period of 41 months (range, 3 to 81 months), liver enzyme levels declined significantly in all patients. Six of 10 patients with follow-up liver biopsies showed histologic improvement. Significant radiographic deterioration was seen in only 1 of 10 patients who had endoscopic retrograde cholangiography. In 7 patients previously treated with UDCA alone, liver enzyme levels declined significantly only after immunosuppressive therapy was added. Adverse drug reactions led to the withdrawal of study medications in 2 patients.

Conclusions: Combined immunosuppressive therapy may alter the progression of primary sclerosing cholangitis. Our observations suggest a benefit from adding immunosuppressive drugs to UDCA therapy. A randomized trial is warranted.

Author and Article Information

From Johannes Gutenberg University, Mainz, Germany.

Requests for Reprints: Ansgar W. Lohse, MD, I. Department of Medicine, Johannes Gutenberg-University, Langenbeckstrasse 1, 55131 Mainz, Germany. For reprint orders in quantities exceeding 100, please contact the Reprints Coordinator; phone, 215-351-2657; e-mail, reprints{at}mail.acponline.org.

Current Author Addresses: Dr. Schirmacher: Department of Pathology, University of Cologne, Josef Stelzmann Strasse 9, 50931 Köln, Germany.

Dr. Gerken: Department of Gastroenterology and Hepatology, University of Essen, Hufelandstrasse 55, 45147 Essen, Germany.

Drs. Schramm, Helmreich-Becker, Meyer zum Büschenfelde, and Lohse: Department of Internal Medicine, University of Mainz, Langenbeckstrasse 1, 55101 Mainz, Germany.