CAJ | 학술논문

目的探讨干燥综合征(SS)多系统损害的临床特点,以便及时、准确诊断,及时治疗,有效减少误诊误治.方法在总结我院诊治1例SS合并血栓性血小板减少性紫癜临床诊治经过的基础上,分析SS合并微血管血栓-出血综合征的临床表现、实验室检查、诊断标准、治疗及相关文献复习.结果 SS合并血栓性血小板减少性紫癜典型的临床表现为血小板减少性紫癜、微血管病性溶血性贫血、中枢神经系统症状、肾脏损伤和发热;治疗上首选血浆置换,同时应用糖皮质激素及免疫抑制剂.结论 SS合并血栓性血小板减少性紫癜临床表现复杂,容易漏诊、误诊,病情进展快,严重威胁患者的生命,病死率高,需予重视.
목적 탐토간조종합정(SS)다계통손해적림상특점,이편급시、준학진단,급시치료,유효감소오진오치.방법 재총결아원진치1례SS합병혈전성혈소판감소성자전림상진치경과적기출상,분석SS합병미혈관혈전-출혈종합정적림상표현、실험실검사、진단표준、치료급상관문헌복습.결과 SS합병혈전성혈소판감소성자전전형적림상표현위혈소판감소성자전、미혈관병성용혈성빈혈、중추신경계통증상、신장손상화발열;치료상수선혈장치환,동시응용당피질격소급면역억제제.결론 SS합병혈전성혈소판감소성자전림상표현복잡,용역루진、오진,병정진전쾌,엄중위협환자적생명,병사솔고,수여중시.
Objective To investigate the clinical characteristics of multisystem impairment of Sj(o)gren's syndrome (SS) to make quick and accurate diagnose and treatment to decrease the rate of misdiagnosis and mistreatment.Methods A case of thrombotic thrombocytopenic purpura with SS was reported and the related literatures were reviewed.The clinical manifestations,laboratory examinations,diagnostic criteria and therapy of thrombotic thrombocytopenic purpura with SS were analyzed.Results Purpura haemorrhagica,hemolytic anemia,central nervous systemic symptoms,kidney impairment and fever were the representative clinical manifestations of thrombotic thrombocytopenic purpura with SS.Plasmapheresis was the most effective therapy with the background treatment with glucocorticosteroid and immunosuppressive agents.Conclusion Thrombotic thrombocytopenic purpura with SS presents a complicated clinical manifestations,so misdiagnosis and mistreatment is very frequent.More attention should be paid to this specific situation because the disease is rare and has high mortality rate.