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Recurrent severe acute hepatitis caused by hypereosinophilic syndrome associated with elevated serum immunoglobulin G4 levels

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Abstract

A 46-year-old male was admitted to our hospital with severe acute hepatitis, hypereosinophilia, and serum immunoglobulin G4 (IgG4) elevation. Plasma exchange was performed, and he was treated by prednisolone; however, his hepatitis recurred twice over the following twelve months. Transjuglar liver biopsy was performed at the third onset, which demonstrated extensive hepatocyte necrosis, congestion, and severe eosinophil infiltration. We diagnosed hypereosinophilic syndrome (HES)-related hepatitis. Although no cholangitis was detected by imaging and pathological diagnosis, IgG4-positive cells were detected in the liver and bone marrow. Furthermore, the elevation of serum IgG4 levels was associated with the eosinophil count and his clinical condition. After the addition of azathioprine to his treatment regimen, no reoccurrence was observed. IgG4-positive cells may have contributed to the severity and refractoriness of this recurrent acute HES-related hepatitis.

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Disclosures

Conflict of Interest:

Tomonori Aoyama, Toshiharu Matsumoto, Akira Uchiyama, Kazuyoshi Kon, Shunhei Yamashina, Satoko Suzuki, Kenichi Ikejima, Takashi Yao, Ryohei Kuwatsuru and Sumio Watanabe declare that they have no conflict of interest.

Human/Animal Rights:

All procedures followed were in accordance with the Helsinki Declaration of 1975, as revised in 2008(5).

Informed Consent:

Informed consent was obtained from the patient for inclusion in the study.

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Correspondence to Tomonori Aoyama.

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Aoyama, T., Matsumoto, T., Uchiyama, A. et al. Recurrent severe acute hepatitis caused by hypereosinophilic syndrome associated with elevated serum immunoglobulin G4 levels . Clin J Gastroenterol 7, 516–522 (2014). https://doi.org/10.1007/s12328-014-0532-0

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  • DOI: https://doi.org/10.1007/s12328-014-0532-0

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