中华临床免疫和变态反应杂志2016，Vol.10Issue(1)：40-45,6.DOI:10.3969/j.issn.1673-8705.2016.01.008
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
摘要
Abstract
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 1st, 1990 to September 30th, 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 (pSS)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 2 patients (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 prognosis is poor with high score of TEN.关键词
结缔组织病/中毒性表皮坏死松解症Key words
connective tissue diseases/toxic epidermal necrolysis分类
医药卫生引用本文复制引用
杨华夏,边赛男,曾月平,赵丽丹,吴庆军,张烜,张奉春..结缔组织病并发中毒性表皮坏死松解症患者临床特点[J].中华临床免疫和变态反应杂志,2016,10(1):40-45,6.基金项目
国家自然科学基金 （）
