Martínez-Barrio J, Ovalles-Bonilla J G, López-Longo F J, González C M, Montoro M, Valor L, Martínez L P, Nieto J C, Hinojosa-Dávila M C, Bello N, Monteagudo I, Naredo E, Carreño L
Department of Rheumatology, Gregorio Marañón General Hospital, Madrid, Spain.
Department of Rheumatology, Gregorio Marañón General Hospital; and Facultad de Medicina, Universidad Complutense de Madrid, Madrid, Spain.
Clin Exp Rheumatol. 2015 Nov-Dec;33(6):788-94. Epub 2015 Nov 17.
This paper aims to identify clinical and serological differences, damage accrual and mortality, in juvenile, adult and late-onset SLE.
We conducted our study with patients fulfilling SLE classification criteria taken from the Hospital Gregorio Marañon Autoimmune Systemic Rheumatic Diseases' Registry (1986 to 2012). Clinical characteristics, laboratory data and therapies used during the course of the disease were analysed with patients divided into 3 groups: juvenile-onset (≤ 18 years), adult-onset (19-50) and late onset (>50 years).
Four hundred and forty-five patients were included. Renal disease and cutaneous manifestations were more frequent in the juvenile-onset group at disease onset. During follow-up, juvenile-onset group presented a higher incidence of renal disease, malar rash, Raynaud's phenomenon, cutaneous vasculitis, and neuropsychiatric manifestations than the other two groups. Arthritis and lymphopoenia were more frequent in the adult-onset group. Arterial hypertension and neoplasm were more frequent in the late-onset group. Low serum complement, anti-dsDNA, anti-U1RNP and anti-Sm antibodies were more common in the juvenile-onset group. Patients with late-onset SLE had more damage accrual. Thirty-seven patients (8.3%) died during the study. All-cause mortality was significantly higher in the late-onset group. Age at disease onset >50 years was an independent risk factor for damage accrual (OR, 2.2; 95%CI, 1.1-4.6; p=0.029) and mortality (OR, 2.6; 95%CI, 1.1-6.3; p=0.03).
We found significant differences in clinical and serological profiles between juvenile, adult and late-onset SLE. The most significant of which was a higher prevalence of neuropsychiatric and renal complications as well as different autoantibody signatures for the juvenile-onset group.
本文旨在确定青少年、成人和晚发型系统性红斑狼疮（SLE）在临床和血清学方面的差异、损伤累积情况及死亡率。
我们对符合SLE分类标准的患者进行了研究，这些患者来自格雷戈里奥·马拉尼翁医院自身免疫性系统性风湿病登记处（1986年至2012年）。将患者分为3组：青少年发病组（≤18岁）、成人发病组（19 - 50岁）和晚发病组（>50岁），分析疾病过程中的临床特征、实验室数据和所用治疗方法。
共纳入445例患者。青少年发病组在疾病发作时肾病和皮肤表现更为常见。在随访期间，青少年发病组肾病、颧部红斑、雷诺现象、皮肤血管炎和神经精神表现的发生率高于其他两组。关节炎和淋巴细胞减少在成人发病组更为常见。动脉高血压和肿瘤在晚发病组更为常见。低血清补体、抗双链DNA、抗U1RNP和抗Sm抗体在青少年发病组更为常见。晚发型SLE患者的损伤累积更多。37例患者（8.3%）在研究期间死亡。晚发病组全因死亡率显著更高。发病年龄>50岁是损伤累积（比值比，2.2；95%置信区间，1.1 - 4.6；p = 0.029）和死亡率（比值比，2.6；95%置信区间，1.1 - 6.3；p = 0.03）的独立危险因素。
我们发现青少年、成人和晚发型SLE在临床和血清学特征方面存在显著差异。其中最显著的是青少年发病组神经精神和肾脏并发症的患病率较高，以及自身抗体特征不同。
