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: Yasumori Izumi, Hayato Takeshita, Yuji Moriwaki, Keiko Hisatomi, Masakazu Matsuda, Natsuki Yamashita, Chieko Kawahara, Yoshika Shigemitsu, Nozomi Iwanaga, Atsushi Kawakami, Hirokazu Kurohama, Daisuke Niino, Masahiro Ito, Kiyoshi Migita
• Journal: Modern rheumatology (Mod Rheumatol) Vol. 27 Issue 1 Pg. 174-177 (Jan 2017) ISSN: 1439-7609 [Electronic] England
• PMID: 25528859 (Publication Type: Journal Article)

Chemical References

• Glucocorticoids
• Immunoglobulin G
• Interleukin-6
• Fluorodeoxyglucose F18
• C-Reactive Protein

Topics

• Autoimmune Diseases (blood, diagnosis, diagnostic imaging, pathology)
• Biopsy
• C-Reactive Protein (metabolism)
• Castleman Disease (blood, diagnosis, diagnostic imaging, pathology)
• Diagnosis, Differential
• Female
• Fluorodeoxyglucose F18
• Glucocorticoids (therapeutic use)
• Humans
• Hypergammaglobulinemia (blood, diagnosis, diagnostic imaging, pathology)
• Immunoglobulin G (blood)
• Interleukin-6 (blood)
• Lymph Nodes (diagnostic imaging, pathology)
• Middle Aged
• Plasma Cells (pathology)
• Positron-Emission Tomography