Yamamoto Motohisa, Yajima Hidetaka, Takahashi Hiroki, Yokoyama Yoshihiro, Ishigami Keisuke, Shimizu Yui, Tabeya Tetsuya, Suzuki Chisako, Naishiro Yasuyoshi, Takano Ken-Ichi, Yamashita Ken, Hashimoto Masato, Keira Yoshiko, Honda Saho, Abe Takashi, Suzuki Yasuo, Mukai Masaya, Himi Tetsuo, Hasegawa Tadashi, Imai Kohzoh, Shinomura Yasuhisa
Department of Gastroenterology, Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine , Sapporo , Japan.
Mod Rheumatol. 2015 Mar;25(2):199-204. doi: 10.3109/14397595.2014.950036. Epub 2014 Aug 27.
Abstract Objective. Immunoglobulin (Ig)G4-related disease (IgG4-RD) is a new disease entity that has only been identified this century. Clinical information is thus lacking. We established the Sapporo Medical University and Related Institutes Database for Investigation and Best Treatments of IgG4-related Disease (SMART) to clarify the clinical features of IgG4-RD and provide useful information for clinicians. Methods. Participants comprised 122 patients with IgG4-related dacryoadenitis and/or sialadenitis (IgG4-DS), representing lacrimal and/or salivary lesions of IgG4-RD, followed-up in December 2013. We analyzed the sex ratio, mean age at onset, organ dysfunction, history or complications of malignancy, treatments, rate of clinical remission, and relapse. Results. The sex ratio was roughly equal. Mean age at diagnosis was 59.0 years. Positron emission tomography revealed that the ratio of other organ involvements was 61.4%. Complications of malignancy were observed in 7.4% of cases. Glucocorticoid was used to treat 92.1% of cases, and the mean maintenance dose of prednisolone was 4.8 mg/day. Rituximab was added in three cases, and showed good steroid-sparing effect. The clinical remission rate was 73.8%, and the annual relapse rate was 11.5%. Half of the cases experienced relapses within 7 years of initial treatment. Conclusion. We analyzed the clinical features and treatments of IgG4-DS using SMART, providing useful information for everyday clinical practice.
摘要 目的。免疫球蛋白（Ig）G4相关疾病（IgG4-RD）是本世纪才被确认的一种新的疾病实体。因此缺乏临床信息。我们建立了札幌医科大学及相关机构IgG4相关疾病调查与最佳治疗数据库（SMART），以阐明IgG4-RD的临床特征，并为临床医生提供有用信息。方法。研究对象为2013年12月随访的122例IgG4相关泪腺炎和/或涎腺炎（IgG4-DS）患者，代表IgG4-RD的泪腺和/或唾液腺病变。我们分析了性别比例、平均发病年龄、器官功能障碍、恶性肿瘤病史或并发症、治疗方法、临床缓解率和复发率。结果。性别比例大致相等。诊断时的平均年龄为59.0岁。正电子发射断层扫描显示其他器官受累比例为61.4%。7.4%的病例观察到恶性肿瘤并发症。92.1%的病例使用糖皮质激素治疗，泼尼松龙的平均维持剂量为4.8毫克/天。3例加用利妥昔单抗，显示出良好的激素节省效果。临床缓解率为73.8%，年复发率为11.5%。一半的病例在初始治疗后7年内复发。结论。我们使用SMART分析了IgG4-DS的临床特征和治疗方法，为日常临床实践提供了有用信息。
