Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain, Carrer Villarroel, 170, 08036 Barcelona, Catalonia, Spain.
Division of Rheumatology, University of Padova, Via Giustiniani 2, 35128 Padova, Italy.
Autoimmun Rev. 2014 Jun;13(6):621-9. doi: 10.1016/j.autrev.2013.11.007. Epub 2014 Jan 10.
To analyse the differences in disease expression of European SLE patients based on gender, age at diagnosis, and ethnicity.
A two-year, retrospective, multicentre, observational study was carried out in five countries (France, Germany, Italy, Spain and the UK). Patients' clinical manifestations including disease activity, organ involvement, organ damage and flares were analysed.
Thirty-one centres enrolled 412 consecutive eligible patients (90.5% of women), with active disease, stratified by disease severity (half severe and half non-severe). Baseline characteristics included; mean (SD) age: 43.3 (13.6) years, SLE duration: 10.7 (8.0) years and age at disease diagnosis: 32.6 (13.0) years old. The mean (SD) SELENA-SLEDAI and SLICC/ACR scores were: 8.1 (6.7) and 0.82 (1.36), respectively. Over half of patients experienced flares (54.9%). The average number of annual flares was 1.01 (0.71) flares/year. In males compared to females, the renal system was more frequently active (53.8% vs 30.0%, p=0.002), the mean SLICC/ACR score was higher (1.15 vs 0.79, p=0.039) and the pulmonary system was more likely to be damaged (12.8% vs 3.8%, p=0.010). Furthermore, patients diagnosed at younger age displayed more renal system activity (young: 56.3% vs adult: 33.4% vs elder: 8.9%, p<0.001) and renal damage (25.0% vs 6.9% vs 2.2%, p=0.018) compared to the others. The annual number of flares (1.13 vs 1.05 vs 0.81 flares/year, p<0.0001), including the occurrence of severe flares (0.58 vs 0.51 vs 0.20, p<0.0001), was also higher in these patients. Conversely, greater organ damage was observed in patients diagnosed at an older age compared to the others. The mean SLICC/ACR score was higher (1.31 vs young: 0.88 and adult: 0.78, p<0.001) in patients diagnosed in the older age groups. The pulmonary (13.3% vs younger: 0% vs adult: 3.7%, p=0.030) and cardiovascular (17.8% vs younger: 0% vs adult: 2.9%, p<0.001) systems were more frequently damaged in these patients. Black African descents showed greater disease activity compared to Caucasian patients. They flared more often (77.1% vs 48.6%, p=0.001) and experienced a greater number of annual flares (1.57 vs 0.89 flares/year, p<0.0001), mainly more severe flares (0.89 vs 0.38/year, p<0.0001). They also were more likely to experience renal system damage.
The study showed clearly two patient subsets. The disease was the most active in Black African descents, and this phenomenon has never been described before in continental Europe. The disease was also more active in patients diagnosed at a younger or adult. Greater disease damage was observed in males and in patients diagnosed at an older age.
分析欧洲 SLE 患者基于性别、诊断时年龄和种族的疾病表现差异。
在五个国家(法国、德国、意大利、西班牙和英国)进行了一项为期两年的回顾性、多中心、观察性研究。分析了患者的临床表现,包括疾病活动度、器官受累、器官损害和发作。
31 个中心共纳入 412 名符合条件的连续患者(90.5%为女性),疾病严重程度分层(一半严重,一半不严重),有活动性疾病。基线特征包括:平均(SD)年龄:43.3(13.6)岁,SLE 病程:10.7(8.0)年,疾病诊断时年龄:32.6(13.0)岁。平均(SD)SELENA-SLEDAI 和 SLICC/ACR 评分分别为:8.1(6.7)和 0.82(1.36)。超过一半的患者出现发作(54.9%)。平均每年发作次数为 1.01(0.71)次/年。与女性相比,男性更常出现肾脏系统活跃(53.8% vs 30.0%,p=0.002),SLICC/ACR 评分更高(1.15 vs 0.79,p=0.039),肺部系统更易受损(12.8% vs 3.8%,p=0.010)。此外,年龄较小的患者更易出现肾脏系统活跃(年轻:56.3% vs 成人:33.4% vs 老年:8.9%,p<0.001)和肾脏损害(25.0% vs 6.9% vs 2.2%,p=0.018)。每年发作次数(1.13 vs 1.05 vs 0.81 次/年,p<0.0001),包括严重发作次数(0.58 vs 0.51 vs 0.20,p<0.0001),在这些患者中也更高。相反,与其他患者相比,年龄较大的患者发生器官损害的可能性更高。SLICC/ACR 评分更高(1.31 vs 年轻:0.88 和成人:0.78,p<0.001)在年龄较大的患者中。肺部(13.3% vs 年轻:0% vs 成人:3.7%,p=0.030)和心血管系统(17.8% vs 年轻:0% vs 成人:2.9%,p<0.001)在这些患者中更易受损。黑人患者比白种人患者表现出更大的疾病活动度。他们更频繁地发作(77.1% vs 48.6%,p=0.001),每年发作次数更多(1.57 vs 0.89 次/年,p<0.0001),主要是更严重的发作(0.89 vs 0.38 次/年,p<0.0001)。他们也更容易出现肾脏系统损害。
研究清楚地显示了两个患者亚组。黑人患者的疾病最为活跃,这种现象在以前的欧洲大陆从未描述过。在年龄较小或成人时诊断的患者中,疾病也更为活跃。男性和年龄较大的患者疾病损害更大。