Masaki Yasufumi, Shimizu Hironori, Sato Nakamura Tomomi, Nakamura Takuji, Nakajima Akio, Iwao Kawanami Haruka, Miki Miyuki, Sakai Tomoyuki, Kawanami Takafumi, Fujita Yoshimasa, Tanaka Masao, Fukushima Toshihiro
Hematology and Immunology, Kanazawa Medical University.
J Clin Exp Hematop. 2014;54(2):95-101. doi: 10.3960/jslrt.54.95.
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.
本综述描述了日本用于诊断和治疗IgG4相关疾病患者的方法。IgG4相关疾病的诊断基于血清IgG4浓度升高和IgG4（+）浆细胞数量增加。将IgG4相关疾病与其他疾病，尤其是恶性肿瘤区分开来非常重要。日本的共识治疗包括初始剂量的泼尼松龙，每天0.5 - 0.6 mg/kg，随后谨慎且逐步减量。大多数患者需要每天5至10 mg的维持治疗。对糖皮质激素难治的患者要么是真正难治，要么是误诊，因此需要重新评估。
