E0039 - Isolated IgG4-Related Sclerosing Cholangitis a Rare Mimicker of Malignant Strictures

Marcel R. Robles, MD¹, Michael Malkowski, MD¹, Guoliang Zheng, MD², Sandeep Krishnan, MBBS, PhD^3
1Tufts University School of Medicine, St. Elizabeth's Medical Center, Boston, MA; ²St. Elizabeth's Medical Center, Tufts University School of Medicine, Boston, MA; ³St. Elizabeth's Medical Center, Tufts University School of Medicine, Brighton, MA

Introduction: IgG4 related sclerosis cholangitis (IgG4-RSC) is the biliary presentation of a systemic condition known as IgG4-related disease (IgG4-RD). It is a rare cause of cholangitis characterized by dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells. It is a multi organ disease with male predominance which is most commonly associated with autoimmune pancreatitis. Isolated IgG4-RSC is exceedingly rare form of this disease often misdiagnosed as cholangiocarcinoma or pancreatic cancer.

Case Description/Methods: A 74-year-old diabetic man presented to the emergency department with complaints of a 44 pound weight loss within the last two months prior to presentation and a 1 month history of generalized pruritus, skin discoloration and pale stool. Exam was notable for scleral icterus, jaundice and no abdominal pain. Laboratory evaluation showed total bilirubin of 14.2 mg/dl, direct bilirubin > 10 mg/dl, AST 61 and ALT 60 with alkaline phosphatase of 328, lipase was normal. CA 19-9 antigen was 212, CEA and AFP were normal. A computed tomography (CT) of abdomen was obtained which showed no pancreatic mass however noted intra and extra-biliary ductal dilation with common bile duct measuring up to 1.8 cm. Magnetic resonance cholangiopancreatography (MRCP) revealed a focal wall thickening involving mid and lower portion CBD causing severe stenosis concerning for malignancy. Endoscopic retrograde cholangiopancreatography (ERCP) with endoscopic ultrasound (EUS) was planned. ERCP demonstrated a proximal dilatation with 2 cm long stricture located at the distal end of bile duct. A sphincteroctomy was performed, biliary tree was swept with 12-15 mm ballon, multiple biopsies were obtained and CBD stent was replaced. Via EUS, a biopsy of CBD was obtained using a trans-duodenal approach. Pathology ultimately revealed plasma cells with IgG4 positive forms. Serum IgG4 was 246 mg/dl. Patient was diagnosed with IgG4 sclerosis cholangitis and he was started on systemic steroids with an adequate response.

Discussion: IgG4-RSC often initially presents as painless jaundice, weight loss or abdominal pain. Our case illustrates challenges in diagnosis given initial strong suspicion for malignancy. A combination of imaging, serological studies and biopsy are needed for diagnosis. Serum IgG4 is a non-specific test however concentration >135 mg/dl is often considered suspicious. Timely diagnosis and treatment with systemic steroids is essential in order to prevent secondary infection or permanent fibrosis.



Disclosures:


Marcel R. Robles, MD¹, Michael Malkowski, MD¹, Guoliang Zheng, MD², Sandeep Krishnan, MBBS, PhD³. E0039 - Isolated IgG4-Related Sclerosing Cholangitis a Rare Mimicker of Malignant Strictures, ACG 2022 Annual Scientific Meeting Abstracts. Charlotte, NC: American College of Gastroenterology.