摘要
目的总结IgG4相关性疾病（IgG4-RD）的临床特点，提高认识，做到早期诊断及合理治疗。方法对在本院住院期间通过病理及临床特征确诊的36例IgG4-RD患者的临床资料进行回顾性分析，并使用IgG4．RD应答指数（RI）进行评估。结果36例患者中男性30例，女性6例，平均发病年龄65．1岁。36例患者均存在活动性病变，其中颌下腺、淋巴结、腹膜后组织是本组病例最常见受累器官。77．7％患者血清IgG4明显升高，44．4％患者血清补体c3明显降低。血清IgG4升高组与正常组相比，RI值更高、受累器官更多（P〈0．01）。血清IgG4浓度与RI明显相关（r=0．737，P〈0．01）。累及肾脏患者低补体血症发生率高于累及其他器官患者（P〈0．01）。28例患者使用糖皮质激素治疗，治疗后RI值、IgG4浓度均明显降低（P〈0．05）。结论IgG4．RDRI和IgG4浓度均可作为IgG4-RD病情活动度和治疗疗效的评估指标；IgC4-RD的诊断应以病理为基础，联合临床特点综合分析。
Objective To explore the clinical characteristics of IgG4-related disease （IgG4- RD） in Chinese by detailed clinicopathological and laboratory assessments. Methods Tile baseline features of 36 patients with biopsy- proven disease were reviewed. The diagnosis was confirmed by pathology review according to consensus diagnostic criteria and clinicopathologic correlation. Disease activity and damage were assessed by the IgG4-RD responder index （RI）. Results Thirty （83.3%） of the patients were male, while six were female, and the average age of onset was 65.1 years. All of the 36 patients had active disease, in which submandibular gland, lymph nodes, retroperitoneal tissue were the most common affected organs in this group of patients. Among 36 patients, 77.7% had elevated serum IgG4 concentrations and 44.4% had hypocomplementemia. Patients with elevated serum IgG4 had a higher RI, a greater number of organs involved （P 〈 0.01 for all comparisons）. The correlation between serum IgG4 level and RI （r=0.737, P 〈 0.01） was stronger than IgG, ESR, CRP and serum complement levels. The incidence of hypocomplementemia in IgG4-RD patients with renal involvement was higher than that in IgG4- RD patients with other organs involvement （P 〈 0.01）. Twenty- eight patients received glucocorticoids therapy, and had lower RI and serum IgG4 concentration after therapy （P 〈 0.05）. Conclusions Both IgG4-RD R1 and IgG4 concentration may be regarded as assessment markers of disease activity and therapeutic effect of IgG4-RD. The diagnosis of IgG4-RD should be supported by histopathology and clinical features.
出处
《中华肾脏病杂志》
CAS
CSCD
北大核心
2016年第4期253-258,共6页
Chinese Journal of Nephrology
基金
国家自然科学基金面上项目（81170653）
