Immunoglobulin G4-associated autoimmune hepatitis with peripheral blood eosinophilia: a case report

Abstract

Background Immunoglobulin G4 (IgG4) associated autoimmune hepatitis (AIH) has been recognized as a type of autoimmune disease that responds to corticosteroid. The diagnosis is based on elevation of the serum IgG4 level, abundance of IgG4 enhanced plasma cell infiltration in the portal region of the liver, and satisfaction of the criteria for “definite AIH” under the revised International Autoimmune Hepatitis Group (IAIHG) scoring system. However, the clinical course of the disease is unclear. Case presentation A 65-year-old man with jaundice and peripheral blood eosinophilia. His IAIHG and simplified score was compatible with definite AIH and his IgG4 level was elevated. Magnetic resonance imaging did not reveal abnormalities in the hepatobiliary system or pancreas. A liver biopsy revealed interface hepatitis with IgG4 positive plasma cell infiltration in the portal region, without evidence of bile duct injury. He responded to 4-week period of induction prednisolone therapy and had no recurring symptoms under maintenance therapy of 5 mg prednisolone during the 3-year follow up. Conclusions This was a rare case that demonstrated an association between IgG4 associated AIH and the presence of peripheral blood eosinophilia.

Immunoglobulin G4 (IgG4)‐related autoimmune hepatitis and IgG4‐hepatopathy: A histopathological and clinical perspective

“…Subsequently, five similar cases were reported not only in Japan but also in Turkey, Thailand, and Korea (Table 2). 9,13–15,31 Characteristics common in these five cases were: (i) typical AIH that satisfied diagnostic criteria for AIH, (ii) high serum IgG4 levels, (iii) histological findings indicating severe infiltration of IgG4 + plasma cells in the liver, and (iv) being highly responsive to steroid therapy, which satisfied the above‐mentioned diagnostic criteria put forward by Umemura and colleagues. It should also be noted that two of the five cases 9,15 developed AIP later on.…”

“…In addition to diagnostic criteria, the nomenclature for this disorder has not been strictly defined (IgG4‐“associated” AIH vs. IgG4‐“related” AIH). Most studies used IgG4‐“associated” AIH, 5,7–9,12–16,30 but only one report 31 and one review 32 used IgG4‐“related” AIH.…”

“…In 2007, another clinical entity of IgG4‐RD in the liver, IgG4‐AIH, and IgG4‐hepatopathy, which is closely associated with IgG4‐AIH and IgG4‐RD, were proposed by Umemura et al 5,6 . Thereafter, several case series of IgG4‐AIH have emerged from Japan, 7–10 other Asian countries, 11–15 and France 16 . Nevertheless, a consensus has yet to be reached on the definition, its association with classical AIH, the diagnostic criteria, or even its nomenclature.…”

Immunoglobulin G4-related Disease with Marked Eosinophilia: A Case Report and Literature Review

“…We herein report the clinical details of a 78-year-old man with IgG4-RD who presented with marked eosinophilia (29,480/μL). Our literature search identified six previous cases of IgG4-RD complicated with eosinophilia (6)(7)(8)(9)(10). Our patient's peripheral blood eosinophil count was the highest among all reported IgG4-RD patients.…”

“…>3,000/μL) is included in the exclusion criteria of the 2019 ACR/EULAR classification criteria for IgG4-RD (1). However, Table summarizes our case and the five previous reports of IgG4-RD patients complicated with marked eosinophilia [6][7][8][9][10]. The patients' ages ranged from 9 to 80 years old, and 4 of the 6 were men.…”