Journal of Yeungnam Medical Science

Case reports

Immunology and Allergy
Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report: Hyun-Je Kim, Young-Hoon Hong; J Yeungnam Med Sci. 2022;39(2):153-160. Published online July 5, 2021; DOI: https://doi.org/10.12701/yujm.2021.01039

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Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

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Striking effectiveness of siltuximab-based treatment in refractory idiopathic multicentric Castleman disease resembling IgG4-related disease
Xing Gong, Weiwei Yan, Feng Zhang, Dongdong Zhang
Journal of Chemotherapy.2025; : 1. CrossRef

Gastroenterology and Hepatology
Anti-nuclear antibody-negative immunoglobulin G4-associated autoimmune hepatitis mimicking lymphoproliferative disorders: Min Kyu Kang, Jung Gil Park, Joon Hyuk Choi; Yeungnam Univ J Med. 2020;37(2):136-140. Published online March 24, 2020; DOI: https://doi.org/10.12701/yujm.2020.00066

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Abstract PDF

Immunoglobulin G4 (IgG4)-associated autoimmune hepatitis (AIH) is a very rare subtype of autoimmune hepatitis and characterized by marked elevated serum IgG and hepatic infiltration of IgG4-expressing plasma cells. Pathologic confirmation of hepatic IgG4-expressing plasma cells is usually required for the final diagnosis of IgG4-associated AIH. Herein, we report the case of a 47-year-old female diagnosed with autoantibody-negative IgG4-associated AIH mimicking lymphoproliferative disorders.

Citations

Citations to this article as recorded by

IgG4‐Associated Autoimmune Hepatitis: a Systematic Review
Chun‐Hsun Liao, Hsu‐Hua Tseng, Ting‐An Shen, Tzu‐Chan Hong, Shih‐Jer Hsu, Jia‐Horng Kao, Chun‐Jen Liu
Journal of Gastroenterology and Hepatology.2026;[Epub] CrossRef
Immunoglobulin G4-related Autoimmune Hepatitis Following Type 1 Autoimmune Pancreatitis: A Case Report and Literature Review
Chiharu Toh, Shinichi Morita, Nobutaka Takeda, Fusako Yamazaki, Kunihiko Yokoyama, Masatoshi Sato, Daisuke Kumaki, Takeshi Sakai, Kazuhiro Funakoshi, Koichi Tsuneyama
Internal Medicine.2025; 64(11): 1659. CrossRef
A clinicopathological study of IgG4-related autoimmune hepatitis and IgG4-hepatopathy
Atsushi Tanaka, Kenji Notohara, Maki Tobari, Masanori Abe, Takeji Umemura, Atsushi Takahashi, Akemi Tsutsui, Takanori Ito, Kohichi Tsuneyama, Atsushi Masamune, Ken-ichi Harada, Hiromasa Ohira, Mitsuhiro Kawano
Journal of Gastroenterology.2025; 60(5): 632. CrossRef
Hepatic Involvement of Diffuse Large B-Cell Lymphoma Mimicking Antinuclear Antibody-Negative Autoimmune Hepatitis Diagnosed by Liver Biopsy
Euna Lee, Min-Kyu Kang, Gabin Moon, Mi-Jin Gu
Medicina.2022; 59(1): 77. CrossRef
Immunoglobulin G4 (IgG4)‐related autoimmune hepatitis and IgG4‐hepatopathy: A histopathological and clinical perspective
Atsushi Tanaka, Kenji Notohara
Hepatology Research.2021; 51(8): 850. CrossRef

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