Abstract：
OBJECTIVE: The aim of this study was to characterize childhood-onset systemic lupus erythematosus (SLE) in two large cohorts from Turkey and the United States.
METHODS: Patients diagnosed with childhood-onset SLE who fulfilled the 1997 American College of Rheumatology classification criteria for SLE from four reference centers in Turkey and the University of Pittsburgh School of Medicine in the United States were included in this study. A comparative analysis was conducted to evaluate the similarities and differences in clinical and laboratory features, damage accrual, and treatment experiences between the two populations.
RESULTS: A total of 174 patients with childhood-onset SLE were included in this study (108 patients from Turkey and 66 patients from the United States). The female-to-male ratio was similar between the two cohorts (∼3:1, p = .73). The median age at diagnosis was 11.67 years (2.19-17.93) in the Turkish cohort and 13.68 years (2.74-17.93) in the U.S. cohort (p < .001). Photosensitivity (45.4% and 21.2%; p = .007) and renal involvement (41.7% and 36.4%; p = .045) were higher in the Turkish cohort. Anti-Ro/SSA (34.8% and 15.7%; p < .001), anti-Sm (59.1% and 19.4%; p < .001), and anti-RNP (47.0% and 14.8%; p < .001) positivity was more frequent in the U.S. cohort. Current use of rituximab (37.9% and 1.9%; p < .001) and belimumab (19.7% and 0%; p < .001) was more prevalent in the U.S. cohort, while the use of cyclophosphamide (often according to the low dose Euro-Lupus protocol) throughout the disease course (24.1% and 4.5%; p < .001) was more frequent in the Turkish cohort. SLICC/ACR Damage Index scores were not different between the two cohorts.
CONCLUSIONS: This study provides detailed clinical and laboratory features of childhood-onset SLE in two independent and geographically divergent cohorts. Our findings suggest an earlier age of disease onset and a higher prevalence of kidney involvement in Turkish patients. Differences in treatment approaches were also noted. However, damage accrual related to SLE does not appear to be different between the two patient populations.
METHODS: Patients diagnosed with childhood-onset SLE who fulfilled the 1997 American College of Rheumatology classification criteria for SLE from four reference centers in Turkey and the University of Pittsburgh School of Medicine in the United States were included in this study. A comparative analysis was conducted to evaluate the similarities and differences in clinical and laboratory features, damage accrual, and treatment experiences between the two populations.
RESULTS: A total of 174 patients with childhood-onset SLE were included in this study (108 patients from Turkey and 66 patients from the United States). The female-to-male ratio was similar between the two cohorts (∼3:1, p = .73). The median age at diagnosis was 11.67 years (2.19-17.93) in the Turkish cohort and 13.68 years (2.74-17.93) in the U.S. cohort (p < .001). Photosensitivity (45.4% and 21.2%; p = .007) and renal involvement (41.7% and 36.4%; p = .045) were higher in the Turkish cohort. Anti-Ro/SSA (34.8% and 15.7%; p < .001), anti-Sm (59.1% and 19.4%; p < .001), and anti-RNP (47.0% and 14.8%; p < .001) positivity was more frequent in the U.S. cohort. Current use of rituximab (37.9% and 1.9%; p < .001) and belimumab (19.7% and 0%; p < .001) was more prevalent in the U.S. cohort, while the use of cyclophosphamide (often according to the low dose Euro-Lupus protocol) throughout the disease course (24.1% and 4.5%; p < .001) was more frequent in the Turkish cohort. SLICC/ACR Damage Index scores were not different between the two cohorts.
CONCLUSIONS: This study provides detailed clinical and laboratory features of childhood-onset SLE in two independent and geographically divergent cohorts. Our findings suggest an earlier age of disease onset and a higher prevalence of kidney involvement in Turkish patients. Differences in treatment approaches were also noted. However, damage accrual related to SLE does not appear to be different between the two patient populations.
摘要：
目的：本研究的目的是在土耳其和美国的两个大型队列中描述儿童期发病的系统性红斑狼疮（SLE）的特征。
方法：本研究包括来自土耳其四个参考中心和美国匹兹堡大学医学院的符合1997年美国风湿病学会SLE分类标准的儿童期发病SLE患者。进行了比较分析，以评估临床和实验室特征的异同，损害应计，和两个人群之间的治疗经验。
结果：本研究共纳入174例儿童发病的SLE患者（108例来自土耳其，66例来自美国）。这两个队列的男女比例相似(~3:1，p=.73)。土耳其队列的诊断年龄中位数为11.67岁（2.19-17.93），美国队列为13.68岁（2.74-17.93）（p<.001）。在土耳其队列中，光敏性（45.4％和21.2％；p=.007）和肾脏受累（41.7％和36.4％；p=.045）较高。抗Ro/SSA（34.8%和15.7%；p<.001），反Sm（59.1%和19.4%；p<.001），抗RNP(47.0%和14.8%;p<.001)阳性在美国队列中更常见。目前使用利妥昔单抗（37.9%和1.9%；p<.001）和贝利木单抗（19.7%和0%；p<.001）在美国队列中更为普遍，而在整个病程中使用环磷酰胺（通常根据低剂量Euro-Lupus方案）（24.1％和4.5％；p<.001）在土耳其队列中更为频繁。SLICC/ACR损伤指数评分在两组之间没有差异。
结论：本研究在两个独立且地理上不同的队列中提供了儿童期发病SLE的详细临床和实验室特征。我们的发现表明，土耳其患者的疾病发病年龄较早，肾脏受累的患病率较高。还注意到治疗方法的差异。然而,与SLE相关的损害累积在两个患者人群之间似乎没有差异。
方法：本研究包括来自土耳其四个参考中心和美国匹兹堡大学医学院的符合1997年美国风湿病学会SLE分类标准的儿童期发病SLE患者。进行了比较分析，以评估临床和实验室特征的异同，损害应计，和两个人群之间的治疗经验。
结果：本研究共纳入174例儿童发病的SLE患者（108例来自土耳其，66例来自美国）。这两个队列的男女比例相似(~3:1，p=.73)。土耳其队列的诊断年龄中位数为11.67岁（2.19-17.93），美国队列为13.68岁（2.74-17.93）（p<.001）。在土耳其队列中，光敏性（45.4％和21.2％；p=.007）和肾脏受累（41.7％和36.4％；p=.045）较高。抗Ro/SSA（34.8%和15.7%；p<.001），反Sm（59.1%和19.4%；p<.001），抗RNP(47.0%和14.8%;p<.001)阳性在美国队列中更常见。目前使用利妥昔单抗（37.9%和1.9%；p<.001）和贝利木单抗（19.7%和0%；p<.001）在美国队列中更为普遍，而在整个病程中使用环磷酰胺（通常根据低剂量Euro-Lupus方案）（24.1％和4.5％；p<.001）在土耳其队列中更为频繁。SLICC/ACR损伤指数评分在两组之间没有差异。
结论：本研究在两个独立且地理上不同的队列中提供了儿童期发病SLE的详细临床和实验室特征。我们的发现表明，土耳其患者的疾病发病年龄较早，肾脏受累的患病率较高。还注意到治疗方法的差异。然而,与SLE相关的损害累积在两个患者人群之间似乎没有差异。
