title: Case of autoimmune hepatitis with markedly enlarged hepatoduodenal ligament lymph nodes.
abstract: Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology. The disease is characterized histologically by interface hepatitis, biochemically by increased aspartate aminotransferase and alanine aminotransferase levels, and serologically by increased autoantibodies and immunoglobulin G levels. Here we discuss AIH in a previously healthy 37-year-old male with highly elevated serum levels of soluble interleukin-2 receptor and markedly enlarged hepatoduodenal ligament lymph nodes (HLLNs, diameter, 50 mm). Based on these observations, the differential diagnoses were AIH, lymphoma, or Castleman's disease. Liver biopsy revealed the features of interface hepatitis without bridging fibrosis along with plasma cell infiltration which is the typical characteristics of acute AIH. Lymph node biopsy revealed lymphoid follicles with inflammatory lymphocytic infiltration; immunohistochemical examination excluded the presence of lymphoma cells. Thereafter, he was administered corticosteroid therapy: after 2 mo, the enlarged liver reached an almost normal size and the enlarged HLLNs reduced in size. We could not find AIH cases with such enlarged lymph nodes (diameter, 50 mm) in our literature review. Hence, we speculate that markedly enlarged lymph nodes observed in our patient may be caused by a highly activated, humoral immune response in AIH.
authors.affiliation: Department of Internal Medicine, Aiseikai Yamashina Hospital, Shichouno-cho, Takehana, Yamashina-ku, Kyoto 602-8086, Japan.
authors.forename: Hideki
authors.initials: H
authors.lastname: Fujii
authors.forename: Naoki
authors.initials: N
authors.lastname: Ohnishi
authors.forename: Kazuho
authors.initials: K
authors.lastname: Shimura
authors.forename: Masafumi
authors.initials: M
authors.lastname: Sakamoto
authors.forename: Tohru
authors.initials: T
authors.lastname: Ohkawara
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Sawa
authors.forename: Koichi
authors.initials: K
authors.lastname: Nishida
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Ohkawara
authors.forename: Tatsuro
authors.initials: T
authors.lastname: Kobata
authors.forename: Kanji
authors.initials: K
authors.lastname: Yamaguchi
authors.forename: Yoshito
authors.initials: Y
authors.lastname: Itoh
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D001706:Biopsy; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006801:Humans; D056724:Immunity, Humoral; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008099:Liver / Q000187:drug effects / Q000276:immunology / Q000473:pathology*; D008198:Lymph Nodes / Q000187:drug effects / Q000473:pathology*; D008206:Lymphatic Diseases / Q000097:blood / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D008297:Male; D064847:Multimodal Imaging / Q000379:methods; D049268:Positron-Emission Tomography; D011237:Predictive Value of Tests; D015375:Receptors, Interleukin-2 / Q000097:blood; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D015375:Receptors, Interleukin-2
keywords: Autoimmune hepatitis; Corticosteroid; Hepatoduodenal ligament lymph nodes; Hepatomegaly; Humoral immune response
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