Abdel-Nabi H H, Abdel-Noor R A
1 Pediatric Department, Tanta University, Tanta, Egypt.
2 Internal Medicine Department, Tanta University, Tanta, Egypt.
Lupus. 2018 May;27(6):1039-1044. doi: 10.1177/0961203318760208. Epub 2018 Feb 20.
Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease that occurs worldwide in both children and adults, with different disease manifestations, activity and severity between them. Objectives To analyse the difference in disease onset patterns and activity in Egyptian children and adults with SLE. Methods A retrospective cohort study conducted on 298 Egyptian SLE patients, 215 adults (a-SLE) (>18 years) and 83 children (j-SLE) (≤18 years). Disease onset, clinical manifestations and laboratory investigations were recorded. The systemic lupus erythematosus disease activity index (SLEDAI) was used to assess disease onset activity; renal biopsy was performed for all cases affected with renal symptoms. Results A total of 215 a-SLE (F/M: 14.4/1), mean age 29.65 ± 10.235 years, and 83 j-SLE (F/M: 5.4/1), mean age at diagnosis 12.63 ± 3.112 years. The most frequent initial a-SLE symptoms were constitutional (88.8%), mucocutaneous (71.2%), haematological (64.2%), articular (62.3%), renal (43.7%), vascular (15.3%), serositis (14.4%) and finally central nervous system (11.6%). There were no significant differences between a-SLE and j-SLE with regard to constitutional, mucocutaneous, renal, vascular and serositis manifestations, which were 92.8%, 74.7%, 53%, 16.9% and 10.8%, respectively, but the j-SLE haematological (88%) and central nervous system (30.1%) manifestations were significantly higher than a-SLE and articular manifestations were significantly lower in j-SLE (14.5%) than a-SLE. Antinuclear antibodies were positive for 95.3% of a-SLE and 97.6% of j-SLE. Anti-dsDNA was positive for 84.7% a-SLE and was significantly higher in j-SLE (92.8%). The SLEDAI score was 12.23 ± 4.966 in a-SLE and was significantly higher in j-SLE (27.13 ± 19.968). International Society of Nephrology lupus nephritis classes III and IV (42.4%) were the commonest findings in a-SLE; however, classes I and III (57.9%) were the commonest in j-SLE. Conclusions SLE had a wide variety of clinical and immunological manifestations, with some similarity and differences between a-SLE and j-SLE; juvenile onset lupus had a higher SLEDAI with more aggressive initial manifestations than a-SLE.
系统性红斑狼疮（SLE）是一种复杂的自身免疫性疾病，在全球儿童和成人中均有发生，两者的疾病表现、活动度和严重程度有所不同。目的：分析埃及儿童和成人SLE患者的发病模式和活动度差异。方法：对298例埃及SLE患者进行回顾性队列研究，其中215例为成人（a-SLE）（>18岁），83例为儿童（j-SLE）（≤18岁）。记录发病情况、临床表现和实验室检查结果。采用系统性红斑狼疮疾病活动指数（SLEDAI）评估发病时的疾病活动度；对所有有肾脏症状的病例进行肾活检。结果：共有215例a-SLE患者（女性/男性：14.4/1），平均年龄29.65±10.235岁，83例j-SLE患者（女性/男性：5.4/1），诊断时平均年龄12.63±3.112岁。成人SLE最常见的首发症状为全身症状（88.8%）、皮肤黏膜症状（71.2%）、血液系统症状（64.2%）、关节症状（62.3%）、肾脏症状（43.7%）、血管症状（15.3%）、浆膜炎（14.4%），最后是中枢神经系统症状（11.6%）。成人SLE与儿童SLE在全身症状、皮肤黏膜症状、肾脏症状、血管症状和浆膜炎表现方面无显著差异，但儿童SLE的血液系统症状（88%）和中枢神经系统症状（30.1%）显著高于成人SLE；儿童SLE的关节症状（14.5%）显著低于成人SLE。抗核抗体在95.3%的成人SLE患者和97.6%的儿童SLE患者中呈阳性。抗双链DNA在84.7%的成人SLE患者中呈阳性，在儿童SLE患者中显著更高（92.8%）。成人SLE的SLEDAI评分为12.23±4.966，在儿童SLE中显著更高（27.13±19.968）国际肾脏病学会狼疮性肾炎Ⅲ级和Ⅳ级（42.4%）是成人SLE最常见的表现；然而，Ⅰ级和Ⅲ级（57.9%）是儿童SLE最常见的表现）。结论：SLE有多种临床和免疫学表现，成人SLE与儿童SLE之间存在一些异同；儿童期发病的狼疮SLEDAI更高，初始表现比成人SLE更具侵袭性。
