Tavangar-Rad Fariba, Ziaee Vahid, Moradinejad Mohammad-Hassan, Tahghighi Fatemeh
Growth and Development Research Center.
Pediatric Rheumatology Research Group, Rheumatology Research Center ; Department of Pediatrics, Tehran University of Medical Sciences ; Children's Medical Center, Pediatrics Center of Excellence, Tehran, Iran.
Iran J Pediatr. 2014 Aug;24(4):365-70. Epub 2014 Jul 1.
Juvenile systemic lupus erythematosus (JSLE) is a common rheumatologic disorder that involves multi organ systems. Prognosis of lupus in children may be poorer than in adults. In this study, we determined mortality and morbidity rates in the pediatric SLE in Iran.
In a cross sectional study, we evaluated 120 children with SLE who had registered in our pediatric rheumatology database within 2004-2010. Data including sex, age, remission, age at the time of diagnosis, age at the time of study, various types of organ involvement, mortality and remission were extracted from this database. Findings : From 120 cases, 77% (92 cases) were females and 23% (28 cases) males (F:M=3.3). Mean follow up period was 56±32 months and mean age at the time of manifesting disease 10.34±2.9 years. Mortality rate was 10% (12 cases) in our investigation. Musculoskeletal involvement showed significant difference between various age groups (P<0.01), that was more frequent in group of 7 years and older. Frequency of oral ulcer and ophthalmic involvement in boys was significantly higher than in girls (P<0.05). Frequency of cardiovascular involvement (P<0.01) and renal involvement (P<0.01) was significantly higher in the patients who had no remission. There was a significant association between mortality rate and cardiac (P<0.02, OR=4.9), pulmonary (P<0.01, OR=10.167) and liver (P<0.05, OR=1.19) involvement.
In our investigation 1-year survival rate was 97% and 5-year survival rate 89%. Liver, cardiac and pulmonary involvements have an association with higher mortality in JSLE patients.
青少年系统性红斑狼疮（JSLE）是一种累及多器官系统的常见风湿性疾病。儿童狼疮的预后可能比成人更差。在本研究中，我们确定了伊朗儿童系统性红斑狼疮（SLE）的死亡率和发病率。
在一项横断面研究中，我们评估了2004年至2010年期间在我们儿科风湿病数据库中登记的120例SLE患儿。从该数据库中提取了包括性别、年龄、缓解情况、诊断时年龄、研究时年龄、各种器官受累类型、死亡率和缓解情况等数据。结果：120例病例中，77%（92例）为女性，23%（28例）为男性（女：男 = 3.3）。平均随访期为56±32个月，发病时的平均年龄为10.34±2.9岁。在我们的调查中，死亡率为10%（12例）。肌肉骨骼受累在不同年龄组之间存在显著差异（P<0.01），在7岁及以上组中更为常见。男孩口腔溃疡和眼部受累的发生率显著高于女孩（P<0.05）。未缓解患者的心血管受累（P<0.01）和肾脏受累（P<0.01）发生率显著更高。死亡率与心脏（P<0.02，OR = 4.9）、肺部（P<0.01，OR = 10.167）和肝脏（P<0.05，OR = 1.19）受累之间存在显著关联。
在我们的调查中，1年生存率为97%，5年生存率为89%。肝脏、心脏和肺部受累与JSLE患者较高的死亡率相关。
