84-Year-Old Male With Obstructive Jaundice Due to IGG-4 Related Sclerosing Cholangitis

Abstract

Patient is 83 y.o. male with history of DM type 2, hypertension, CAD, was hospitalized with 2 weeks of progressive fatigue and jaundice, bilirubin 11.2 mg/dL; ALT 227 U/L and AST 150 U/L, alkaline phosphatase 842 U/L and normal CA 19-9 U/mL. CT abdomen showed intra and extrahepatic biliary ductal dilatation with abrupt tapering of the common bile duct (CBD) at the level of the pancreatic head, prominent pancreas. ERCP revealed normal pancreatic duct and distal CBD stricture; pancreatic and biliary stents were placed, biopsies of the stricture were non diagnostic. Repeat ERCP 1 month later showed resolution of distal biliary stricture and formation of new strictures in the CBD, left hepatic duct and intrahepatic bile ducts. CA 19-9 was mildly elevated at 44 U/mL. Strictures were dilated and additional stricture biopsies were obtained. Pathology showed mild increase in numbers of IgG4 plasma cells up to 28 IgG4 positive plasma cells per 400 per high power field (HPF). Liver biopsy showed a dense peribiliary plasma cell-rich inflammatory infiltrate in a setting of fibrotic background, variable presence of eosinophils within the infiltrate, absence of prominent lymphocytic infiltration of the biliary epithelium, canalicular cholestasis without significant ductular proliferation; mild sinusoidal lymphoplasmacytic infiltrate. An immunohistochemical stain for IgG4 showed numerous peribiliary plasma cells (over 70 IgG4-expressing plasma cells per 400x microscopic field), compatible with IgG4-associated cholangitis. (Figure 1 and 2) IgG4 level was 214.2 mg/dL. Patient was started on 40 mg daily of prednisone which lead to resolution of symptoms and normalization of liver tests. Discussion: IgG4-related disease is an increasingly recognized immune-mediated condition which is comprised of a collection of disorders that share particular pathologic, serologic, and clinical features. It has slight predominance in middle age and older males as we see in our case. IgG4 — related sclerosing cholangitis ((IgG4-SC) may present as a distal or proximal-type disease with or without autoimmune pancreatitis. In some cases diagnosis may be challenging. It can mimic primary sclerosing cholangitis and cholangiocarcinoma due to elevation in CA 19-9 level. High serum immunoglobulin G4 (IgG4 level) is an important feature. Liver biopsy can aid in the prompt diagnosis. High level of suspicion and awareness of this condition can lead to prompt diagnosis and correct treatment.1251_A.tif Figure 1: No Caption available.1251_B.tif Figure 2: No Caption available.