Clinicopathological features of autoimmune hepatitis with IgG4-positive plasma cell infiltration

AimsAlthough liver biopsy is crucial to diagnose and guide treatment decisions, a detailed histological analysis of autoimmune hepatitis (AIH) with clinically acute presentations has not yet been performed. This study aimed to characterise the histological features and explore potential histological hallmarks to diagnose the acute presentation of AIH.MethodsWe systematically evaluated liver specimens of 87 adult patients with acute presentation of AIH retrospectively enrolled from Japanese multicentre facilities. Each histological feature was predefined by consensus based on the diagnostic criteria.ResultsKey findings were that acute presentation of AIH revealed histological features of both acute hepatitis and chronic hepatitis accompanying various degrees of fibrosis. The prominent features were lobular necrosis/inflammation (97.7%), plasma cell infiltration (96.4%), emperipolesis (89.3%), pigmented macrophages (84.5%), cobblestone appearance of hepatocytes (82.6%) and perivenular necroinflammatory activity, including centrilobular necrosis (81.4%).ConclusionsThe acute presentation of AIH represents the entire histological spectrum of acute hepatitis and chronic hepatitis with various activity grades and fibrosis stages that clinically correspond to acute-onset AIH and acute exacerbation of classic AIH, respectively. Although there are no pathognomonic features for the pathological diagnosis, the prominent presence of lobular and perivenular necroinflammatory activity, pigmented macrophages and cobblestone appearance of hepatocytes in addition to the classic AIH features, such as plasma cell infiltration and emperipolesis, are useful for the pathological diagnosis of the acute presentation of AIH.

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

BMC Gastroenterology ◽

10.1186/s12876-020-01559-7 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Arunchai Chang ◽

Cheep Charoenlap ◽

Keerati Akarapatima ◽

Attapon Rattanasupar ◽

Varayu Prachayakul

Keyword(s):

Autoimmune Hepatitis ◽

Plasma Cell ◽

Peripheral Blood ◽

Cell Infiltration ◽

Immunoglobulin G4 ◽

Blood Eosinophilia ◽

Plasma Cell Infiltration ◽

Portal Region ◽

Peripheral Blood Eosinophilia ◽

Igg4 Level

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.

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