Chang Arunchai, Charoenlap Cheep, Akarapatima Keerati, Rattanasupar Attapon, Prachayakul Varayu
Division of Gastroenterology, Department of Internal Medicine, Hatyai Hospital, Songkhla, Thailand.
Department of Anatomical Pathology, Hatyai Hospital, Songkhla, Thailand.
BMC Gastroenterol. 2020 Dec 11;20(1):420. doi: 10.1186/s12876-020-01559-7.
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.
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.
This was a rare case that demonstrated an association between IgG4 associated AIH and the presence of peripheral blood eosinophilia.
免疫球蛋白G4（IgG4）相关自身免疫性肝炎（AIH）已被确认为一种对皮质类固醇有反应的自身免疫性疾病。诊断基于血清IgG4水平升高、肝脏门区IgG4增强的浆细胞浸润丰富，以及符合修订后的国际自身免疫性肝炎小组（IAIHG）评分系统下的“明确AIH”标准。然而，该疾病的临床病程尚不清楚。
一名65岁男性，伴有黄疸和外周血嗜酸性粒细胞增多。他的IAIHG和简化评分符合明确AIH，且IgG4水平升高。磁共振成像未显示肝胆系统或胰腺有异常。肝脏活检显示界面性肝炎，门区有IgG4阳性浆细胞浸润，无胆管损伤证据。他对4周的诱导性泼尼松龙治疗有反应，在3年随访期间，泼尼松龙5mg维持治疗下无复发症状。
这是一例罕见病例，显示了IgG4相关AIH与外周血嗜酸性粒细胞增多之间的关联。
