title: Multicentric Castleman disease mimicking IgG4-related disease: A case report.
abstract: A 50-year-old woman was referred to our hospital for shoulder joint stiffness. She had a history of polyclonal hypergammaglobulinemia and an elevated C-reactive protein level. Her laboratory data revealed an elevated serum immunoglobulin G4 (IgG4) level, hypergammaglobulinemia, and rheumatoid factor positivity in the absence of anticyclic citrullinated peptide antibody. [18F]-Fluorodeoxyglucose positron emission tomography showed significant [18F]-fluorodeoxyglucose uptake in multiple lymph nodes (axillary, hilar, para-aortic, and inguinal). Biopsy of the inguinal lymph node showed expansion of the interfollicular areas by heavily infiltrating plasma cells, consistent with multicentric Castleman disease (MCD). Immunohistochemical analysis revealed a 37.3% IgG4-positive:IgG-positive plasma cell ratio, indicating overlapping IgG4-related disease. However, serological cytokine analysis revealed elevated levels of interleukin-6 (9.3 pg/ml) and vascular endothelial growth factor (VEGF) (1210 pg/ml), which are compatible with MCD. Corticosteroid treatment resolved the serological and imaging abnormalities. IgG4-related disease can mimic MCD, and it is crucial to distinguish between these two diseases. Serum interleukin-6 and VEGF levels may help to discriminate MCD from IgG4-related disease.
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Yasumori
authors.initials: Y
authors.lastname: Izumi
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Hayato
authors.initials: H
authors.lastname: Takeshita
authors.affiliation: b Departments of Hematology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Yuji
authors.initials: Y
authors.lastname: Moriwaki
authors.affiliation: c Departments of Respiratory Medicine , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Keiko
authors.initials: K
authors.lastname: Hisatomi
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Masakazu
authors.initials: M
authors.lastname: Matsuda
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Natsuki
authors.initials: N
authors.lastname: Yamashita
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Chieko
authors.initials: C
authors.lastname: Kawahara
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Yoshika
authors.initials: Y
authors.lastname: Shigemitsu
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Nozomi
authors.initials: N
authors.lastname: Iwanaga
authors.affiliation: d First Department of Internal Medicine , Nagasaki University Graduate School of Biomedical Sciences , Nagasaki , Japan.
authors.forename: Atsushi
authors.initials: A
authors.lastname: Kawakami
authors.affiliation: e Departments of Pathology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Kurohama
authors.affiliation: e Departments of Pathology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Daisuke
authors.initials: D
authors.lastname: Niino
authors.affiliation: e Departments of Pathology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Masahiro
authors.initials: M
authors.lastname: Ito
authors.affiliation: a Departments of General Internal Medicine and Rheumatology , Nagasaki Medical Center , Omura City, Nagasaki , Japan.
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Migita
mesh_terms: D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D001706:Biopsy; D002097:C-Reactive Protein / Q000378:metabolism; D005871:Castleman Disease / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D019788:Fluorodeoxyglucose F18; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006942:Hypergammaglobulinemia / Q000097:blood / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000473:pathology; D007074:Immunoglobulin G / Q000097:blood*; D015850:Interleukin-6 / Q000097:blood; D008198:Lymph Nodes / Q000000981:diagnostic imaging / Q000473:pathology; D008875:Middle Aged; D010950:Plasma Cells / Q000473:pathology; D049268:Positron-Emission Tomography
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D015850:Interleukin-6; D019788:Fluorodeoxyglucose F18; D002097:C-Reactive Protein
keywords: IgG4-related disease; Interleukin-6; Multicentric Castleman disease