Journal of Clinical and Experimental Hematopathology
Online ISSN : 1880-9952
Print ISSN : 1346-4280
ISSN-L : 1346-4280
Review Article
IgG4-Related Disease: Diagnostic Methods and Therapeutic Strategies in Japan
Yasufumi MasakiHironori ShimizuTomomi Sato NakamuraTakuji NakamuraAkio NakajimaHaruka Iwao KawanamiMiyuki MikiTomoyuki SakaiTakafumi KawanamiYoshimasa FujitaMasao TanakaToshihiro Fukushima
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2014 Volume 54 Issue 2 Pages 95-101


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]

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© 2014 by The Japanese Society for Lymphoreticular Tissue Research
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