3008 Painless Jaundice, Sclerosing Cholangitis, and IgG-4 Related Disease

Abstract

INTRODUCTION: IgG4-RD can mimic PSC and Cholangiocarcinoma when presenting in the hepatobiliary system, and should be placed high on the differential diagnosis when there is multi-organ system involvement and negative workup for malignancy. CASE DESCRIPTION/METHODS: A 58 year-old male presented to the ED with painless jaundice and 6 months of episodic RUQ pain, cervical lymphadenopathy, increased anorexia, weight loss, and fatigue. Prior work-up included a CT abdomen/pelvis that showed intra-hepatic ductal dilation and possible stricture/mass at the common hepatic duct, as well as FNA biopsies of lymph nodes negative for malignancy. Initial lab work was significant for T bili of 8.5, AST of 289, AST of 231, and alkaline phosphatase of 1033, and lipase of 26. Physical exam was notable for scleral icterus, cervical lymphadenopathy, and jaundiced skin. Patient was admitted and underwent ERCP with findings of strictures with PSC-like beading; a stent was placed with improvement in his biliary obstruction. Brushings were obtained for cytology, which were negative for malignancy. Given concern for malignancy, further evaluation was performed including tumor markers which revealed an elevated CA 19-9. Staging chest CT revealed adenopathy concerning for metastases. Subsequent excisional biopsy of an enlarged cervical node was negative for malignancy but revealed a non-caseating granuloma. A second ERCP revealed an inflammatory stricture with brushings again negative for malignancy. Given lack of evidence of malignancy, an IgG4 level was checked which was elevated at 386. His presentation was thus thought to be most consistent with IgG4 sclerosing cholangitis, and he was started on systemic corticosteroids. DISCUSSION: IgG4-RD is characterized as a chronic relapsing fibro-inflammatory syndrome. Increased serum levels of IgG4 and infiltration of tissue by IgG4+ plasma cells are typical of IgG4-RD. Common histological findings include dense lymphoplasmacytic infiltrate with IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. The above serological and histological findings together make a diagnosis of IgG4-RD likely. IgG4-RD can occur in multiple organ systems, with typical presentation in the pancreatico-hepatobiliary system as Autoimmune Pancreatitis or Autoimmune Sclerosing Cholangitis. Steroids are first-line treatment, with refractory cases requiring more aggressive immunosuppressive agents, such as Rituximab, to decrease the recurrence of inflammation seen in patients.