Sakamoto Ana Paula, Silva Clovis Artur, Saad-Magalhães Claudia, Alencar Aline Nicácio, Pereira Rosa Maria Rodrigues, Kozu Katia, Barbosa Cassia Maria Passarelli Lupoli, Terreri Maria Teresa
Universidade Federal de São Paulo (UNIFESP).
Faculdade de Medicina da Universidade de São Paulo (USP).
Acta Reumatol Port. 2017 Jul-Sep;42(3):250-255.
To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients.
Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA.
SJS and TEN was observed in 5/852 (0.6%) cSLE female patients, three patients were classified as SJS and two patients were classified as overlap SJS-TEN; TEN was not observed. The mean duration of SJS and overlap SJS-TEN was 15 days (range 7-22) and antibiotics induced four cases. Regarding extra-cutaneous manifestations, hepatomegaly was observed in two cSLE patients, nephritis in two and neuropsychiatric involvement and conjunctivitis were observed respectively in one patient. Hematological involvement included lymphopenia in four, leucopenia in three and thrombocytopenia in two patients. The mean SLEDAI-2K score was 14.8 (range 6-30). Laboratory analysis showed low C3, C4 and/or CH50 in two patients and the presence of anti-dsDNA autoantibody in two patients. One patient had lupus anticoagulant and another one had anticardiolipin IgG. All patients were treated with steroids and four needed additional treatment such as intravenous immunoglobulin in two patients, hydroxychloroquine and azathioprine in two and intravenous cyclophosphamide in one patient. Sepsis was observed in three cSLE patients. Two patients required intensive care and death was observed in one patient.
Our study identified SJS and overlap SJS-TEN as rare manifestations of active cSLE associated with severe multisystemic disease, with potentially lethal outcome.
评估大量儿童期起病的系统性红斑狼疮（cSLE）患者中的史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）。
多中心研究，纳入了巴西圣保罗儿科风湿病中心随访的852例cSLE患者。SJS定义为体表脱落面积低于体表面积（BSA）的10%，重叠型SJS-TEN为10%-30%，TEN为大于30%的BSA。
在5/852（0.6%）例cSLE女性患者中观察到SJS和TEN，3例患者被分类为SJS，2例患者被分类为重叠型SJS-TEN；未观察到TEN。SJS和重叠型SJS-TEN的平均病程为15天（范围7-22天），4例由抗生素诱发。关于皮肤外表现，2例cSLE患者出现肝肿大，2例出现肾炎，1例分别出现神经精神受累和结膜炎。血液系统受累包括4例淋巴细胞减少、3例白细胞减少和2例血小板减少。SLEDAI-2K评分的平均值为14.8（范围6-30）。实验室分析显示2例患者C3、C4和/或CH50降低，2例患者存在抗双链DNA自身抗体。1例患者有狼疮抗凝物，另1例有抗心磷脂IgG。所有患者均接受了类固醇治疗，4例患者需要额外治疗，2例患者接受静脉注射免疫球蛋白，2例患者接受羟氯喹和硫唑嘌呤，1例患者接受静脉注射环磷酰胺。3例cSLE患者发生败血症。2例患者需要重症监护，1例患者死亡。
我们的研究确定SJS和重叠型SJS-TEN是活动性cSLE的罕见表现，与严重的多系统疾病相关，具有潜在的致命后果。
