Boddaert Jacques, Huong Du Le Thi, Amoura Zahir, Wechsler Bertrand, Godeau Pierre, Piette Jean-Charles
From Department of Internal Medicine, Groupe hospitalier Pitié-Salpêtrière, Paris, France.
Medicine (Baltimore). 2004 Nov;83(6):348-359. doi: 10.1097/01.md.0000147737.57861.7c.
Systemic lupus erythematosus (SLE) is uncommon after the age of 50 years, and studies of elderly patients with SLE are scarce. We conducted the current study to analyze characteristics and outcome of patients with late-onset SLE in a French tertiary referral center, and to compare them with those of younger patients with SLE. From 1980 to 2000, 47 patients were identified as having late-onset SLE, defined as SLE diagnosed at or over the age of 50 years. These patients were compared with a group of 114 randomly selected patients aged younger than 50 years at SLE diagnosis. We compared clinical characteristics, laboratory data, therapy, and course. The female to male ratio was smaller in the late-onset SLE group (p = 0.0012). Some manifestations occurred less frequently in late-onset SLE: arthritis (p = 0.009), malar rash (p = 0.013), and nephropathy (p = 0.009). High-dose corticosteroids (p = 0.0016) and immunosuppressive drugs (p = 0.006) were less commonly used in the elderly. Deaths occurred more frequently in late-onset SLE (p = 0.019), with a 10-year survival rate of 71% versus 95% in early-onset SLE (p < 0.01). In patients with late-onset SLE, causes of death were usually unrelated to SLE. Analysis of pooled data from the literature, based on 714 old and 4700 young SLE patients, confirmed that late-onset SLE was characterized by a smaller female to male ratio (4.4:1 vs. 10.6:1; p = 3.10); a higher occurrence of serositis (36.7% vs. 28.6%; p = 7.10) and pulmonary involvement (21.2% vs. 11.3%; p = 6.10); and a lower occurrence of malar rash (31.1% vs. 62.4%; p = 10), photosensitivity (26.2% vs. 38.2%; p = 6.10), purpura/cutaneous vasculitis (13.4% vs. 25.9%; p = 9.10), alopecia/hair loss (24% vs. 44.9%; p = 3.10), Raynaud phenomenon (24.8% vs. 37.2%; p = 3.10), neuropsychiatric manifestations (15.3% vs. 20.2%; p = 0.025), lymphadenopathy (9.1% vs. 19.6%; p = 2.10), nephrotic syndrome (8.1% vs. 24.3%; p = 0.015), and nephritis (28.6% vs. 42.7%; p = 2.10). Regarding laboratory features, rheumatoid factor positivity was more frequent (32.7% vs. 20.1%; p = 3.10), whereas anti-RNP positivity (10.4% vs. 20.9%; p = 9.10), anti-Sm positivity (9.1% vs. 17.1%; p = 0.001), and a low CH50 complement fraction (45% vs. 64.9%; p = 0.002) were less frequent in old compared with young SLE patients. In conclusion, the clinical pattern of late-onset SLE is characterized by a lower disease severity. The reduced survival observed in this group seems to result mainly from the consequences of aging.
系统性红斑狼疮（SLE）在50岁之后并不常见，针对老年SLE患者的研究也很少。我们开展了本研究，以分析法国一家三级转诊中心中迟发性SLE患者的特征和预后，并将其与年轻SLE患者进行比较。1980年至2000年期间，47例患者被确诊为迟发性SLE，定义为在50岁及以上确诊的SLE。这些患者与一组114例在SLE诊断时年龄小于50岁的随机选择患者进行比较。我们比较了临床特征、实验室数据、治疗方法和病程。迟发性SLE组的男女比例较小（p = 0.0012）。一些表现较少见于迟发性SLE：关节炎（p = 0.009）、颧部红斑（p = 0.013）和肾病（p = 0.009）。老年患者较少使用大剂量皮质类固醇（p = 0.0016）和免疫抑制药物（p = 0.006）。迟发性SLE患者的死亡更为常见（p = 0.019），10年生存率为71%，而早发性SLE为95%（p < 0.01）。在迟发性SLE患者中，死亡原因通常与SLE无关。基于714例老年和4700例年轻SLE患者的文献汇总数据分析证实，迟发性SLE的特征为男女比例较小（4.4:1 vs. 10.6:1；p = 3.10）；浆膜炎（36.7% vs. 28.6%；p = 7.10）和肺部受累（21.2% vs. 11.3%；p = 6.10）的发生率较高；而颧部红斑（31.1% vs. 62.4%；p = 10）、光过敏（26.2% vs. 38.2%；p = 6.10）、紫癜/皮肤血管炎（13.4% vs. 25.9%；p = 9.10）、脱发（24% vs. 44.9%；p = 3.10）、雷诺现象（24.8% vs. 37.2%；p = 3.10）、神经精神表现（15.3% vs. 20.2%；p = 0.025）、淋巴结病（9.1% vs. 19.6%；p = 2.10）、肾病综合征（8.1% vs. 24.3%；p = 0.015）和肾炎（28.6% vs. 42.7%；p = 2.10）的发生率较低。关于实验室特征，类风湿因子阳性更为常见（32.7% vs. 20.1%；p = 3.10），而与年轻SLE患者相比，老年患者中抗RNP阳性（10.4% vs. 20.9%；p = 9.10）、抗Sm阳性（9.1% vs. 17.1%；p = 0.001）和低CH50补体分数（45% vs. 64.9%；p = 0.002）的情况较少见。总之，迟发性SLE的临床模式特征为疾病严重程度较低。该组观察到的生存率降低似乎主要是衰老的结果。
