结缔组织病并发中毒性表皮坏死松解症患者临床特点  

Clinical Features of Patients with Connective Tissue Diseases Complicated with Toxic Epidermal Necrolysis

作　　者：杨华夏[1] 边赛男 曾月平[1,2] 赵丽丹[1] 吴庆军[1] 张烜[1] 张奉春[1] 

机构地区：[1]中国医学科学院,北京协和医学院,北京协和医院风湿免疫科、风湿免疫病学教育部重点实验室,北京100730 [2]中国医学科学院,北京协和医学院,北京协和医院皮肤科,北京100730

出　　处：《中华临床免疫和变态反应杂志》2016年第1期40-45,共6页Chinese Journal of Allergy & Clinical Immunology

基　　金：国家自然科学基金(81541037)

摘　　要：目的探讨结缔组织病(connective tissue diseases,CTD)并发中毒性表皮坏死松解症(toxic epidermal necrolysis,TEN)患者的临床特点。方法回顾性分析1990年1月至2015年9月北京协和医院确诊CTD并发TEN住院患者的临床资料,分析其临床特点、实验室检查、治疗及结局。并与同期国内外报道的CTD并发TEN病历资料进行比较。结果 CTD并发TEN住院患者共7例,女性6例,男性1例,中位年龄42岁。7例CTD中系统性红斑狼疮3例(42.9%)、多发性肌炎1例(14.3%)、原发性干燥综合征1例(14.3%)、未分化结缔组织病2例(28.6%)。7例TEN发作前均有药物诱因,药物至TEN发作中位时间3 d。药物种类包括:解热镇痛药3例(42.9%)、抗癫痫药2例(28.6%)、抗生素2例(28.6%)、抗病毒药物1例(14.3%)。CTD活动度,发生TEN时6例(85.7%)CTD病情稳定,仅1例(14.3%)疾病活动。7例患者中ANA阳性6例(85.7%),抗SSA阳性4例(57.1%)。大剂量激素及免疫球蛋白治疗后5例(71.4%)好转,2例(28.6%)死亡(死亡原因为重症感染)。TEN评分高者预后差。结论并发TEN的CTD患者以SLE多见,多由药物诱发,疾病本身不活动。大剂量激素及免疫球蛋白治疗有效,TEN评分高者预后差。Objective To investigate the clinical features of patients diagnosed of connective tissue diseases（ CTD） complicated with toxic epidermal necrolysis（ TEN）. Methods We retrospectively analyzed the clinical manifestations, laboratory examinations, treatments and outcomes of patients diagnosed of connective tissue diseases complicated with toxic epidermal necrolysis in Peking Union Medical College Hospital from January 1-（st）, 1990 to September 30-（th）, 2015, and compared with cases reported internationally in the same period. Results We found 7 patients diagnosed with CTD complicated with TEN with complete data in total. There were 6 females and 1 male. The median age was 42 years old. The primary diagnosis included systemic lupus erythematosus（ SLE） in 3 cases（ 42. 9%）,polymyositis（ PM） in 1 case（ 14. 3%）,primary Sjogren Syndrome（ p SS） in 1 case（ 14. 3%）,and undifferentiated connective tissue diseases（ UCTD） in 2 cases（ 28. 6%）. All patients＇ TENs were triggered by drugs,the median time from taking the possible drug to onset of TEN was 3 days,and the possible drugs included 3 cases of nonsteroidal anti-inflammatory drugs（ NSAIDS）（ 42. 9%）,2 cases of antiepileptic（ 28. 6%）,2 cases of antibiotics（ 28. 6%）,and 1 case of antiviral drug（ 14. 3%）. When TENs occurred,connective tissue diseases were not active in six patients（ 85. 7%）, and active in 1 patient（ 14. 3%）. Six patients（ 85. 7%） had positive ANA,and 4 patients had positive anti-SSA（ 57. 1%）. After treatment with large dose of glucocorticoids and intravenous immunoglobulins（ IVIG）,5 patients（ 71. 4%） recovered and 2patients（ 28. 6%） died of severe infection. The prognosis was poor in patients with high score of TEN. Conclusions SLE is the common connective tissue diseases in patients complicated with TEN. Most TENs are triggered by drugs,and the connective tissue diseases are not active. Treatment of high dose of glucocorticoids and IVIG is effective,and the progn

关 键 词：结缔组织病 中毒性表皮坏死松解症 

分 类 号：R593.2[医药卫生—内科学]