P1234 - Demographics and serologic data of a group of IgG4-related hepatobiliary disease patients

Gregory Capelli, DO, Rehana Begum, MD, Eitan Scheinthal, DO, Meklit Hunde, MD, Adil Ghafoor, MD, Andrew Foong, MD
Aurora Healthcare, Milwaukee, WI

Introduction: IgG4-related disease (IgG4-RD) is an immune-mediated multiorgan fibroinflammatory condition which can manifest as hepatic disease, a condition termed IgG4-related hepatobiliary disease (IgG4-HD). This condition commonly affects the biliary tree, termed IgG4-related sclerosing cholangitis (IgG4-SC) or presents with isolated hepatic involvement, termed IgG4-related hepatopathy. IgG4-HD is incompletely understood and can lead to progressive fibrosis and cirrhosis. IgG4-HD is rare in IgG4-RD, which itself is a rare condition. For this reason, there is a paucity of data regarding demographic and serologic data of IgG4-HD in the United States. The goal of this study was to describe demographics and diagnostic data of patients with IgG4-HD across a large healthcare system.

Methods: Following IRB approval, the electronic medical record was queried to identify patients with the ICD-4 code for IgG4-RD (D89.84) presenting between 1/2011 and 1/2023. Manual chart review was used to identify patients with IgG4-HD, to collect demographic information, diagnostic information, and laboratory values.

Results: In total, 13 patients were identified. Among the group, 10 were males, 9 were white, 9 were non-Hispanic, and 1 was African American. The average age at diagnosis of 56.4 ±14.3 years old, and average body mass index at diagnosis of 25.7±5.1kg/m2. Among this group, 11 patients had an IgG4 level drawn at presentation, with 9 having an IgG4 of over 140mg/dL. Further lab data from the patient’s time of diagnosis is displayed in figure 1. All required a pathologic tissue sample for diagnosis. In the group, 2 presented with a liver mass and 4 presented with an isolated biliary stricture. The group included 5 patients who were first diagnosed with autoimmune hepatitis (AIH), and 3 patients who were first diagnosed with primary sclerosing cholangitis (PSC), before the diagnoses were revised to IgG4-HD. The majority of the patients (10) were diagnosed with IgG4-SC.

Discussion: Among this group of IgG4-HD patients, most had an elevated IgG on serology, and were white, non-Hispanic males. Most presented with IgG-SC, suggesting that this condition often involves the biliary tree. In total, 8 patients (61%) were diagnosed with either PSC or AIH before the diagnosis was later changed to IgG4-HD, suggesting that this disease is often misidentified. Due to the rarity of this condition, further studies will be needed to better describe these findings.


Disclosures:


Gregory Capelli, DO, Rehana Begum, MD, Eitan Scheinthal, DO, Meklit Hunde, MD, Adil Ghafoor, MD, Andrew Foong, MD. P1234 - Demographics and serologic data of a group of IgG4-related hepatobiliary disease patients, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.