A Rare Presentation of IgG4-Cholangitis without Evidence of Autoimmune Pancreatitis

Walaa Hammad¹, Qirat Jawed², Islam Younes², Ana Romero², Jesus Romero^2, , Asnia Latif² and Fareeha Abid²

¹Pathology Department, Ain Shams University hospital, Cairo, Egypt

²Internal Medicine Department, RWJBarnabasHealth/Trinitas Regional Medical Center, Elizabeth, NJ, USA

Cite this paper

Walaa Hammad, Qirat Jawed, Islam Younes, Ana Romero, Jesus Romero, Asnia Latif and Fareeha Abid. A Rare Presentation of IgG4-Cholangitis without Evidence of Autoimmune Pancreatitis. American Journal of Medical Case Reports. 2022; 10(6):170-172. doi: 10.12691/AJMCR-10-6-2

Abstract

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a clinical entity recognized as a biliary manifestation within the spectrum of the systemic disorder known as IgG4-related disease (IgG4-RD). The prevalence of this disease is frequently underestimated since it can be misdiagnosed as cholangiocarcinoma or primary sclerosing cholangitis. 90% of patients present with coexistent autoimmune pancreatitis (AIP), while only 10% of the patients present with isolated biliary involvement. Here, we present a rare case of a 54-year-old gentleman who presented to our hospital with abdominal pain and cholestatic liver injury found to have IgG4-SC in the absence of pancreatitis. IgG4-SC represents a chronic inflammatory state which is usually seen in the fifth and sixth decades of life. These patients frequently present with symptoms characterized by obstructive jaundice, pruritus, and abdominal discomfort. Approximately 90% of the patients will have an elevation of serum IgG4 levels (≥140 mg/dL). Characteristic imagining findings include thickening of common bile duct wall, narrowing of the long segments and dilation of the upstream biliary system, and no evidence of biliary masses. Although it is frequently associated with autoimmune pancreatitis, it can present as an isolated disease. Hence, IgG4 sclerosing cholangitis should be part of the differential diagnosis of patients presenting with biliary strictures. It is of utmost importance to differentiate IgG4-SC from its frequent mimickers such as primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and pancreato-biliary malignancies since therapeutic strategies and outcomes differ significantly between these clinical entities. Glucocorticoids are the first-line treatment and immunomodulatory drugs are used in patients with disease relapse after steroid therapy, which is seen in 50-60% of cases.

Keywords

IgG4 sclerosing cholangitis, primary sclerosing cholangitis, autoimmune pancreatitis

Copyright

This work is licensed under a Creative Commons Attribution 4.0 International License. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/

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