目的:探讨男性儿童系统性红斑狼疮(SLE)的临床特点。方法:收集2006年1月至2015年8月住院的41例男性SLE患儿的临床资料,与同期204例女性患儿中年龄匹配的41例进行临床资料比较分析。结果:两组患儿在起病年龄、籍贯分布、家族史、首发临床表现、炎性指标及免疫学指标方面比较差异无统计学意义(P>0.05)。两组患儿的常见首发临床表现均为发热、皮疹、浮肿和关节肌肉疼痛。组内对比发现活动期红细胞沉降率(ESR)较C反应蛋白(CRP)的阳性率更高(P<0.05)。男女两组间蛋白尿、肾功能不全、血液系统损害、心脏损害和神经精神狼疮的发生率比较差异无统计学意义(P>0.05)。28例男性和20例女性完善肾活检,两组病理分型均以Ⅳ型及以上为主。治疗上均以糖皮质激素和免疫抑制剂为主要药物。以SLEDAI评分系统对患者的转归进行评价,好转率男87.8%(36例),女82.9%(34例),差异无统计学意义;病死率男4.9%(2例),女12.2%(5例),差异无统计学意义。7例死亡患儿的起病年龄为(8.0±1.9)岁,显著低于总样本82例患儿的起病年龄的(11.2±2.5)岁(P<0.05)。结论:男性SLE患儿以发热、皮疹、浮肿和关节肌肉疼痛为常见首发表现,肾脏、血液、心脏、神经精神系统容易受累,狼疮肾炎以病理Ⅳ型及以上为主,起病年龄越小可能病情更危重。 Objective: To investigate the clinical features of male children with systemic lupus erythematosus (SLE). Methods: The clinical data of 41 male SLE children hospitalized from January 2006 to August 2015 were collected, and compared with the age-matched 41 cases of 204 female children in the same period. Results: There was no significant difference between the two groups in onset age, distribution of native origin, family history, initial clinical manifestations, inflammatory indexes and immunological indexes (P> 0.05). Common clinical manifestations in both groups were fever, rash, edema and joint and muscle pain. The intra-group comparison found that the active erythrocyte sedimentation rate (ESR) was higher than C-reactive protein (CRP) positive rate (P <0.05). There was no significant difference in the incidence of proteinuria, renal insufficiency, blood system damage, heart damage and neuropsychiatric lupus between men and women (P> 0.05). Twenty-eight male and 20 female perfected renal biopsy. The pathological types of the two groups were mainly type Ⅳ and above. Treatment are glucocorticoid and immunosuppressive agents as the main drug. The rate of improvement was 87.8% (36 cases) and 82.9% (34 cases) in the SLEDAI scoring system. The difference was not statistically significant; the mortality rate was 4.9% in male (2 cases) and 12.2% in female 5 cases), the difference was not statistically significant. The onset age of 7 deaths was (8.0 ± 1.9) years, significantly lower than the onset age of 82 children (11.2 ± 2.5) years (P <0.05). CONCLUSIONS: The incidence of fever, rash, edema and joint and muscle pain are common in male children with SLE. Kidney, blood, heart and neuropsychiatric system are prone to be affected. Lupus nephritis is characterized by pathological type Ⅳ and above, and the younger age of onset may be The condition is more critical.