St. Joseph's Regional Medical Center Paterson, NJ, United States
Ariana R. Tagliaferri, MD, BS, Heemani Ruparel, BS, Gabriel Melki, MD, BS, Yana Cavanagh, MD, FACG, Matthew Grossman, MD, FACG St. Joseph's Regional Medical Center, Paterson, NJ
Introduction: Autoimmune Cholangitis (AIC) is a rare immune-mediated condition with an annual incidence of 1.4/100,000. The majority of cases are associated with pancreatitis and are often incidentally discovered in the workup of acute or chronic pancreatitis. Only 8% account for isolated IgG4 AIC. Herein we present a rare case of IgG4 AIC in the absence of pancreatitis.
Case Description/Methods: A 68 yo M PMH DM, HTN, HLD and BPH presented with acute RUQ abdominal pain, objective fevers, fatigue and anorexia. On arrival, he was hemodynamically stable and afebrile. Exam was remarkable for jaundice, epigastric and RUQ pain without rigidity or guarding and no evidence of organomegaly. Labs were remarkable for leukocytosis with left shift, normocytic anemia, hepatocellular-mixed cholestatic elevation in LFT’s with unconjugated hyperbilirubinemia. He was admitted for acute cholangitis and treated with IV Zosyn. CT Abdomen/Pelvis showed no acute abnormalities, however MRCP revealed intrahepatic ductal dilatation and a transition point near common hepatic duct. ERCP was performed and stents were placed in the common hepatic and pancreatic ducts. He was discharged however returned for persistent abdominal pain. Upon further workup, IgG4 was elevated (400) and liver biopsy revealed extensive histological and pathological findings compatible with autoimmune cholangitis. The patient was successfully treated with IV steroids and antibiotics, and was subsequently discharged. On clinical follow up, the patient was asymptomatic with normalized bilirubin levels.
Discussion: The pathogenesis of AIC is poorly understood; however, IgG4 causes infiltration of T cells, leading to progressive fibrosis and organ damage which can affect any organ system. Commonly, this pathological inflammation is seen in the pancreas and biliary tract concurrently due to the hepatopancreatic lymphatic and hematogenous drainage. Other less commonly affected organs include the kidney, liver, salivary and lacrimal glands. Early recognition of IgG4 acute cholangitis is crucial as its presentation can mimic pancreatic cancer, hepatobiliary malignancies, PSC or other infectious causes of acute cholangitis. The broad differentials warrant vastly different treatments and have varying long term consequences.
Disclosures: Ariana Tagliaferri indicated no relevant financial relationships. Heemani Ruparel indicated no relevant financial relationships. Gabriel Melki indicated no relevant financial relationships. Yana Cavanagh indicated no relevant financial relationships. Matthew Grossman indicated no relevant financial relationships.
Ariana R. Tagliaferri, MD, BS, Heemani Ruparel, BS, Gabriel Melki, MD, BS, Yana Cavanagh, MD, FACG, Matthew Grossman, MD, FACG. P1087 - A Rare Case of Isolated IG4 Autoimmune Cholangitis, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.