An unusual overlap syndrome in a male with chronic active hepatitis

Abstract

A 27 y|[sol]|o African-American male with an insidious history of jaundice and general malaise for one year was referred to us for further work-up and treatment. The patient complained of easy fatigability, and generalized arthralgias for the past 6 months, and had been taking Ibuprofen on a PRN basis. Physical examination showed a palpable spleen, but was otherwise unremarkable. Labs were notable for AST 64 IU|[sol]|L, ALT 105 IU|[sol]|L, Alk. Phos 360, Total Protein 8.4 g|[sol]|dl, Gamma Globulin 27.1%, PT 15.9|[sol]|INR 1.29, WBC 4.0 K|[sol]|cc, Hgb 14|[sol]|Hct 42, PLT 43 k|[sol]|cc |[plus]|ANA 1:320 homogeneous, |[plus]|Anti Smooth Muscle AB 1:160, negative AntiLKM AB, negative Antimitochondrial AB, Iron 128, TIBC 412, negative Hepatitis A|[sol]|B|[sol]|C serology, normal copper and ceruloplasmin levels. A CT scan revealed a homogeneous liver without duct dilation. He underwent a liver biopsy which showed chronic active hepatitis with early features of cirrhosis and was scheduled for an ERCP. At this time he was started on Prednisone 20 mg|[sol]|d Folic acid 1 mg|[sol]|d, OsCal 500 mg TID. The ERCP demonstrated multiple short irregular strictures with beading irregularities of the intrahepatic ducts. At this time Ursodeoxycolic acid 250 mg TID was added.