Multicentric Castleman disease mimicking IgG4-related disease: A case report.

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Multicentric Castleman disease mimicking IgG4-related disease: A case report.

Mod Rheumatol. 2017 Jan;27(1):174-177

Authors: Izumi Y, Takeshita H, Moriwaki Y, Hisatomi K, Matsuda M, Yamashita N, Kawahara C, Shigemitsu Y, Iwanaga N, Kawakami A, Kurohama H, Niino D, Ito M, Migita K

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. [18(F)]-Fluorodeoxyglucose positron emission tomography showed significant [18(F)]-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.

PMID: 25528859 [PubMed - indexed for MEDLINE]

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