The patient reports mild fatigue and pruritis but denies jaundice, scleral icterus, abdominal swelling. What’s your impression?
Patient history. The patient reports some fatgue and pruritis but no evidence of jaundice or abdominal swelling. He is well managed on mesalamine and his chronic colitis has recently been determined to be inactive.
Laboratory, imaging, and biospsy results. Results of laboratory studies confirm liver function abnormalities and findings of MRCP are normal. Livery biopsy demonstrates periductal sclerosis on histology.
What is the next step in management?
A. Refer for expedited liver transplant evaluation
B. Supportive care
C. Initiate prednisone
D. Initiate ursodeoxycholic acid
Answer: B. Supportive care. The diagnosis is primary sclerosing cholangitis (PSC); results of MRCP are normal so there is no target for treatment. Prognosis of small-duct disease is better than for large duct-risk for malignancies is lower. There is no drug treatment for PSC other than supportive care and screening.
No transplant, no drug therapy. Liver transplant is not indicated nor is prednisone appropriate given the +absence of antismooth muscle antibody. The patient is also not a candidate for ursodeoxycholic acid treatment.
Follow Dr James on Twitter: @TedWJamesMDRecommended Reading
Eaton JE, Talwalkar JA, Lazaridis KN, et al. Pathogenesis of primary sclerosing cholangitis and advances in diagnosis and management. Gastroenterol. 2013;145:521-536.
The patient reports mild fatigue and pruritis but denies jaundice, scleral icterus, abdominal swelling. Click through the slides for more patient history and lab results. What’s your diagnosis?