Torrente-Segarra Vicenç, Salman Monte Tarek Carlos, Rúa-Figueroa Iñigo, Sánchez-Alonso Fernando, López-Longo Francisco Javier, Galindo-Izquierdo María, Calvo-Alén Jaime, Olivé-Marqués Alejandro, Ibañez-Ruán Jesús, Horcada Loreto, Sánchez-Atrio Ana, Montilla Carlos, Melero González Rafael Benito, Díez-Álvarez Elvira, Martinez-Taboada Victor, Andreu José Luis, Fernández-Berrizbeitia Olaia, Hernández-Beriain José Ángel, Gantes Marian, Hernández-Cruz Blanca, Pecondón-Español Ángela, Marras Carlos, Bonilla Gema, Pego-Reigosa José M
Department of Rheumatology, Hospitalet-Sant Joan Despí Moisès Broggi University General Hospital, Hospitalet Llobregat, Spain.
Department of Rheumatology, Parc de Salut Mar-IMIM, Barcelona, Spain.
Clin Exp Rheumatol. 2017 Nov-Dec;35(6):1047-1055. Epub 2017 Jun 12.
We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database.
Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years.
We reviewed 3,428 aSLE patients (89.6% women) and 484 jSLE patients (89.8% girls), 93% Caucasian (both groups). Mean age at diagnosis was 38.1±14 and 16.6±6.3 years (p<0.001) and mean age at the end of follow-up was 48.8±14.3 and 31.5±30 years (p<0.001), respectively. jSLE showed significantly more clinical (including lymphadenopathy, fever, malar rash, mucosal ulcers, pericarditis, pleuritis, Raynaud's phenomenon, lupus nephritis, recurrent nephritis, histologic nephritis changes, thrombocytopenia, haemolytic anaemia, thrombotic thrombocytopenic purpura, seizures, lupus headache and organic brain syndrome) and immunological (a-dsDNA and a-Sm antibodies, hypocomplementaemia) involvement than did aSLE, except for secondary Sjögren's syndrome, a-Ro antibodies, fibromyalgia and osteoporosis. jSLE also showed more SLE family history, longer diagnosis delay, higher SLEDAI and Katz scores, but lower Charlson scores than aSLE. Several specific domains were more frequently involved in SLICC/ACR DI in jSLE. jSLE patients more frequently underwent all SLE-related treatment and procedures, as well as dialysis and kidney transplantations.
jSLE shares many clinical and serological features with aSLE. However, jSLE patients typically manifested more activity, severity, cumulative damage in certain areas, than their aSLE counterparts.
我们旨在描述青少年起病的系统性红斑狼疮（jSLE）的特征，并通过一个大型国家数据库确定其与成人起病的系统性红斑狼疮（aSLE）的差异。
分析西班牙风湿病学会狼疮登记处（RELESSER）纳入的患者（≥4条美国风湿病学会标准）的数据。收集社会人口统计学、临床、血清学、活动度、治疗、累积损伤、合并症及严重程度数据。描述发病年龄<18岁的患者，并与发病年龄≥18岁的患者进行比较。
我们回顾了3428例aSLE患者（89.6%为女性）和484例jSLE患者（89.8%为女孩），两组93%为白种人。诊断时的平均年龄分别为38.1±14岁和16.6±6.3岁（p<0.001），随访结束时的平均年龄分别为48.8±14.3岁和31.5±30岁（p<0.001）。jSLE在临床（包括淋巴结病、发热、蝶形红斑、黏膜溃疡、心包炎、胸膜炎、雷诺现象、狼疮性肾炎、复发性肾炎、组织学肾炎改变、血小板减少、溶血性贫血、血栓性血小板减少性紫癜、癫痫发作、狼疮性头痛和器质性脑综合征）和免疫学（抗双链DNA和抗Sm抗体、补体低下）方面的受累情况比aSLE更显著，但继发性干燥综合征、抗Ro抗体、纤维肌痛和骨质疏松症除外。jSLE的SLE家族史也更多，诊断延迟更长，SLE疾病活动指数（SLEDAI）和Katz评分更高，但Charlson评分低于aSLE。在jSLE中，几个特定领域在系统性红斑狼疮国际协作临床/美国风湿病学会损伤指数（SLICC/ACR DI）中受累更频繁。jSLE患者更频繁地接受所有与SLE相关的治疗和操作，以及透析和肾移植。
jSLE与aSLE有许多临床和血清学特征相同。然而，jSLE患者通常比aSLE患者表现出更多的活动度、严重程度和某些方面的累积损伤。
