129例IgG4相关性疾病患者临床特征分析

Clinical characteristics of 129 patients with IgG4 related diseases

目的 分析IgG4相关性疾病(IgG4-RD)患者临床特征和诊治经验,探讨血清IgG4水平、外周血嗜酸性粒细胞水平、治疗方式与预后的关系。方法 收集2013年1月—2020年12月于四川大学华西医院诊断为IgG4-RD的129例患者的临床资料,回顾性分析其临床表现、受累器官、实验室检查结果、治疗经过以及预后情况等。结果 (1)129例患者中,男女性别比为1.74:1,男性和女性的中位年龄分别为56.00(48.75,67.00)岁和50.00(37.00,56.00)岁。(2)眼肿胀和淋巴结肿大是最常见的首发症状,最易受累的器官是淋巴结(尤以头颈部淋巴结多见)、眼睑及泪腺。(3)血清IgG4水平和外周血嗜酸性粒细胞水平升高分别是多器官受累和复发的危险因素。(4)单用糖皮质激素治疗和联用糖皮质激素及免疫抑制剂治疗IgG4-RD都有明显的效果,两种治疗方式的复发率分别为13.64%及23.88%,差异无统计学意义(P>0.05),可能与联用糖皮质激素及免疫抑制剂治疗患者病情更为严重有关。(5)对于部分情况特殊的IgG4-RD患者(压迫重要器官、不可逆性纤维化等)需行手术治疗,但不能降低复发率。结论 IgG4-RD好发于中老年男性,可累及全身多个器官组织,血清IgG4水平升高可能是多器官受累的危险因素,而外周血嗜酸性粒细胞水平升高则可能是复发的危险因素,但有待进一步的大样本研究证实,糖皮质激素和免疫抑制剂都是IgG4-RD的有效治疗手段,部分情况下也可考虑手术,临床上应根据患者实际情况进行抉择。

Objective To analyze the clinical characteristics,diagnosis and treatment experience of patients with IgG4 related diseases(IgG4-RD),and explore the relationship between clinical features(including serum IgG4 level,peripheral blood eosinophil level,treatment)and prognosis.Methods The clinical data of patients diagnosed with IgG4-RD in West China Hospital of Sichuan University from January 2013 to December 2020 were collected,and their clinical manifestations,involved organs,laboratory examination results,treatment and prognosis were analyzed retrospectively.Results Among the 129 patients enrolled,the sex ratio of male to female was 1.74:1,and the median age of men and women were 56.00(48.75,67.00)and 50.00(37.00,56.00)years,respectively.Eye swelling and lymphadenopathy were the most common symptoms,and the most vulnerable organs include lymph nodes(especially located in head and neck),eyelids and lacrimal glands.High serum IgG4 levels and elevated peripheral blood eosinophils were risk factors for multiple organ involvement and recurrence,respectively.Treatment with Glucocorticoids or the combination of glucocorticoids and immunosuppressants both had effects on IgG4-RD,while there is no difference in the recurrence rate of the two treatment methods(13.64%vs 23.88%,P=0.19),which may be due to the more serious condition in patients treated with combination of glucocorticoid and immunosuppressant.For some special cases with IgG4-RD(such as compression of important organs,irreversible fibrosis,etc.),surgical treatment is available without significant reduced recurrence rate.Conclusion IgG4-RD tend to occur in middle-aged and elderly men and can involve multiple organs and tissues.The high level of serum IgG4 and the elevated level of peripheral blood eosinophils may be risk factors for multiple organ involvement and recurrence respectively,which needs to be confirmed by further large sample research.Glucocorticoids and immunosuppressants both are advisable treatment for IgG4-RD,while surgery can also be considered in some cases which should be determined individually.