以紫癜为首发症状的原发性干燥综合征6例临床分析

目的分析以紫癜为首发临床表现的原发性干燥综合征(pSS)患者的临床特征,提高临床医生对本病的认识。方法回顾性分析本科2006年1月-2011年6月收治的6例以紫癜为首发表现的原发性干燥综合征患者的临床资料,包括首诊、临床症状、实验室检查、系统表现、治疗结果及随访情况。结果 6例均被误诊,首诊病程均未见记录眼睛干涩及口干症状,实验室检查结果见结缔组织病样特征,予个体化免疫抑制治疗10d～4周后患者皮疹均消退,血IgG治疗前为(2366.67±754.63)mg/dL,治疗后下降至(1936.67±322.22)mg/dL,总体疾病活动指数也下降,仅1例在药物减量后皮疹复发。结论 pSS患者出现紫癜样皮疹易被误诊,全面了解本病特征,结合典型结缔组织实验室检查结果即可确诊本病。本病可能系高球蛋白所致,抗过敏治疗效果欠佳,个体化规律免疫抑制效果佳。

A Clinical Analysis of Six Cases Suffering from Primary Sjogren's Syndrome with Purpura as Initial Presentation

Objective By retrospective clinical analysis of primary Sjogren’s syndrome with purpura as the initial presentation in 6 cases, to provide a reference for clinical diagnosis and treatment. Methods The clinical data,related laboratory tests,treatment and follow-up data of the 6 cases were collected. Results Six patients all had misdiagnosised,dryness of mouth and eyes, other system complications and different laboratory examinations could be helpful to making correct diagnosis.After treated with individualized immunosuppressive therapy and observated for 10 days~4 weeks,the rash subsided, IgG decreased from(2 366.67±754.63)mg/dL to (1 936.67±322.22)mg/dL,index of disease activity also decreased. The disease has less recurrence.Conclusion The onset of purpura in pSS is easy to make misdiagnosis or missed diagnosis,more clinical material and laboratory examinations should be in consideration.The mechanism may be due to the high globulin. Anti-allergy treatment is ineffective, the law of immunosuppressive therapy is easy to control.