IgG4-Related Disease: Diagnostic Methods and Therapeutic Strategies in Japan

Yasufumi Masaki; Hironori Shimizu; Tomomi Sato Nakamura; Takuji Nakamura; Akio Nakajima; Haruka Iwao Kawanami; Miyuki Miki; Tomoyuki Sakai; Takafumi Kawanami; Yoshimasa Fujita; Masao Tanaka; Toshihiro Fukushima

doi:10.3960/jslrt.54.95

Review Article

IgG4-Related Disease: Diagnostic Methods and Therapeutic Strategies in Japan

Yasufumi Masaki, Hironori Shimizu, Tomomi Sato Nakamura, Takuji Nakamura, Akio Nakajima, Haruka Iwao Kawanami, Miyuki Miki, Tomoyuki Sakai, Takafumi Kawanami, Yoshimasa Fujita, Masao Tanaka, Toshihiro Fukushima

著者情報

キーワード: glucocorticoid, rituximab, ¹⁸FDG-PET, lymphoma, multicentric Castleman's disease

ジャーナルフリー

2014 年 54 巻 2 号 p. 95-101

DOI https://doi.org/10.3960/jslrt.54.95

詳細

抄録

This review describes methods utilized in Japan to diagnose and treat patients with IgG4-related disease. A diagnosis of IgG4-related disease is based on elevated serum IgG4 concentration and an increased number of IgG4⁺ plasma cells. Differentiating IgG4-related disease from other disorders, especially malignancy, is quite important. Consensus treatment in Japan consists of an initial dose of prednisolone at 0.5-0.6 mg/kg/day, followed by careful and gradual dose reduction. Most patients require maintenance treatment at 5 to 10 mg/day. Patients refractory to glucocorticoids are either truly refractory or have been misdiagnosed, therefore requiring reassessment. [J Clin Exp Hematop 54(2) : 95-101, 2014]

責任著者(Corresponding author)

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