Immunoglobulin G4‐associated autoimmune hepatitis later complicated by autoimmune pancreatitis: A case report

• Published in: Hepatology research Vol. 46; no. 6; pp. 601 - 606
• Main Authors: Ishizu, Yoji, Ishigami, Masatoshi, Kuzuya, Teiji, Honda, Takashi, Hayashi, Kazuhiko, Nakano, Isao, Hirooka, Yoshiki, Goto, Hidemi
• Format: Journal Article
• Language: English
• Published: Netherlands 01.05.2016
• Subjects: autoimmune hepatitis; autoimmune pancreatitis; immunoglobulin G4; autoimmune hepatitis; autoimmune pancreatitis; immunoglobulin G4
• ISSN: 1386-6346; 1872-034X
• DOI: 10.1111/hepr.12593

Immunoglobulin G4 (IgG4)‐associated autoimmune hepatitis (AIH) is a new disease entity with elevated levels of serum IgG4 and marked IgG4 positive plasma cell infiltration of the liver, and its clinical course remains unknown. A patient with IgG4‐associated AIH who later developed autoimmune pancreatitis (AIP) is reported. A 73‐year‐old man was admitted to our hospital due to elevated liver transaminase levels, hypergammaglobulinemia and positive antinuclear antibody. A liver biopsy specimen showed severe interface hepatitis with marked lymphoplasmacytic infiltration without damage to the interlobular bile ducts, and a diagnosis of AIH was made. Abdominal computed tomography showed no abnormalities in the pancreas. Prednisolone therapy normalized the transaminase levels. Two years later, the patient developed AIP, which recurred after 5 years due to a reduction in the prednisolone dose. Three years later, he had a recurrence of AIH after discontinuation of prednisolone treatment. Evaluation of serum IgG4 levels and IgG4‐bearing plasma cell infiltration of the liver at both the onset and recurrence of AIH showed that the serum IgG4 levels were 284 and 208 mg/dL, respectively, and the IgG4‐bearing plasma cell infiltration levels were 30–40 cells/high‐power field (HPF) per portal area and 4–10 cells/HPF per portal area, respectively. From these results, this case was finally diagnosed as IgG4‐associated AIH. The course of this patient demonstrates two important clinical lessons: (i) IgG4‐associated AIH can later be complicated by AIP; and (ii) discontinuation of prednisolone treatment can cause recurrence of IgG4‐associated AIH.