Mount Sinai Medical Center Miami Beach, FL, United States
Anna Willet, MD1, Nathaly Cortez, MD1, Yohelys Lobaton, MD2, Francesca Giubilei, MD3, Manuel Berzosa, MD1 1Mount Sinai Medical Center, Miami Beach, FL; 2Universidad Centrooccidental Lisandro Alvarado, Miami Beach, FL; 3Universidad Iberoamericana (UNIBE), Miami, FL
Introduction: IgG4-related disease (IRD) is a fibroinflammatory entity that can affect multiple organ systems and is characterized by abundant infiltration of IgG4+ cells. IgG4-related sclerosing cholangitis (ISC) can be found as an organ manifestation in IRD either in isolation or associated with other organs. Its diagnosis can often be challenging as it is mostly indistinguishable from other entities like primary sclerosing cholangitis (PSC), cholangiocarcinoma (CC) and pancreatic cancer based on cholangiographic findings alone. Given its mimicry of other biliary pathologies on imaging, the clinical history and affection of other organs are crucial to guide the diagnosis.
Case Description/Methods: A 79-year-old female was referred to our Gastroenterology department after an MRI of the pancreas with Magnetic Retrograde Cholangiopancreatography protocol revealed marked biliary ductal dilatation and abrupt cut-off of the common bile duct (CBD) near the pancreatic neck/body; worrisome for neoplasm. Laboratory data revealed elevated AST 90 U/L, ALT 147 U/L, alkaline phosphatase 626 U/L. Rest of the workup was unremarkable. Further anamnesis revealed a history of chronic unexplained submandibular and periorbital swelling, evident on physical exam. We proceeded with an Endoscopic Ultrasound (EUS). The CBD was found to be dilated up to 16 mm. There was marked intrahepatic duct dilation, however, a distinct mass was not clearly seen in the pancreas. Fine needle aspiration biopsies showed nonspecific inflammation but no malignant cells, suggestive of Autoimmune Pancreatitis (AP). Serum IgG4 levels were high at 318 mg/dl (N: 2-96 mg/dl). She was treated with eight weeks of oral corticosteroids, which led to resolution of the swelling of the mandibular and periorbital areas. A repeat CT scan after treatment showed improvement of biliary ductal dilatation. Her liver enzymes and IgG-4 levels decreased. She remained asymptomatic and was monitored with periodic MRI and IgG4 levels.
Discussion: ISC is an entity characterized by infiltration of IgG4+ plasma cells and extensive fibrosis in the bile duct wall. It can present as abdominal pain or obstructive jaundice and is usually associated with AP. It is very important to differentiate ISC from PSC, CC and secondary causes. Diagnosis of ISC is multidisciplinary and includes typical biliary imaging findings; elevated serum IgG4 concentrations; coexistence of IRDs and characteristic histopathological features. Use of corticosteroids typically leads to rapid disease remission.
Figure: Fig. a. Periorbital swelling, pre-treatment. Fig. b. Periorbital swelling, post-treatment. Fig. c. Submandibular swelling, pre-treatment. Fig. d. Submandibular swelling, post-treatment. Fig. e. MRCP showing CBD dilatation up to 2.2 cm, pre-treatment. Fig. f. CT abdomen and pelvis with contrast showing improvement in CBD dilatation to 1.8 cm, post-treatment.
Anna Willet indicated no relevant financial relationships.
Nathaly Cortez indicated no relevant financial relationships.
Yohelys Lobaton indicated no relevant financial relationships.
Francesca Giubilei indicated no relevant financial relationships.
Manuel Berzosa indicated no relevant financial relationships.
Anna Willet, MD1, Nathaly Cortez, MD1, Yohelys Lobaton, MD2, Francesca Giubilei, MD3, Manuel Berzosa, MD1. P2161 - IgG4-Related Sclerosing Cholangitis: A Case Report, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.