原发性干燥综合征288例临床分析

Clinical analysis of 288 patients with primary sjǒgren syndrome

目的探讨原发性干燥综合征(primary sjǒgren syndrome,p SS)患者的临床特征及治疗效果。方法回顾性分析288例p SS患者的临床资料。结果 227例无血小板减少患者(A组)口干、眼干、猖獗齿、关节痛、脾大发生率及血沉值、抗SSB阳性率均低于61例血小板减少组(B组)。B组17例患者行骨髓穿刺,1例骨髓增生低下,16例骨髓增生活跃,15例巨核细胞成熟障碍,2例淋巴细胞增多。B组主要使用糖皮质激素及免疫抑制剂治疗,无效者予甲泼尼龙冲击、静脉丙种球蛋白治疗,1例患者使用利妥昔单抗,1例使用促血小板生成素。结论伴血小板减少的p SS患者更易出现口干、眼干、猖獗齿、关节痛、脾大,且血沉值及抗SSB阳性率更高,治疗上主要使用糖皮质激素及免疫抑制剂,无效者可考虑激素冲击、利妥昔单抗等治疗。

Objective To investigate the clinical feature and therapeutic effect of primary Sjǒgren syndrome（ pSS） patients. Methods The clinical profiles of 288 pSS patients were retrospectively analyzed.Results The incidence of xerostomia,xerophthalmi-a,rampant caries,arthralgia and splenomegaly as well as erythrocyte sedimentation rate（ ESR） and positive rate of anti-SSB in 277 pa-tients without thrombocytopenia（group A）were lower than that in 61 patients with thrombocytopenia（group B）.In the group B,17 pa-tients received bone marrow aspiration.Of the 17 patients,one patient showed bone marrow hyperplasia while 16 cases showed bone marrow hyperplasia.There were 15 patients with megakaryocytic maturation disorder and 2 patients with lymphocytosis.The group B was mainly treated with glucocorticoids and immunosuppressive agents.Non-responders were further given methylprednisolone pulse treat-ment and gamma globulin IV therapy.One patient took rituximab and one patient used thrombopoietin.Conclusion The pSS patients with thrombocytopenia are more likely to present symptoms such as xerostomia,xerophthalmia,rampant caries,arthralgia and splenomeg-aly as well as have higher erythrocyte sedimentation rate and anti-SSB positive rate.The main drugs included glucocorticoids and immu-nosuppressive agents.The methylprednisolone pulse treatment and rituximab treatment should be considered for non-responders.