Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA.
J Rheumatol. 2011 Jul;38(7):1317-25. doi: 10.3899/jrheum.100956. Epub 2011 Jun 17.
Although patients who develop systemic sclerosis (SSc) later in life (≥ 65 yrs) may express the entire clinical spectrum of disease, we hypothesize that patients with late-age onset SSc incur a different risk for specific organ manifestations of disease compared to those with early-age onset SSc.
In total, 2300 patients with SSc were evaluated between 1990 and 2009 and reviewed from a university-based scleroderma center cohort. Demographic profile, SSc subtype, autoantibody status, Medsger severity scores, pulmonary function tests, echocardiography, and right heart catheterization measures were compared between late-age onset vs younger-age onset patients with SSc.
Overall, 2084 patients (91%) developed SSc prior to age 65, while 216 (9%) were ≥ 65 years. Late-age onset patients had a significantly higher proportion of anticentromere antibodies (42% vs 27%; p = 0.001) compared to early-age onset patients. Risk of pulmonary hypertension (OR 1.76, 95% CI 1.00, 3.12), muscle weakness (OR 1.85, 95% CI 1.30, 1.64), renal impairment (OR 2.83, 95% CI 1.98, 4.04), and cardiac disease (OR 2.69, 95% CI 1.92, 3.78) was greater among those with late-age onset SSc; although risk of digital ischemia (OR 0.64, 95% CI 0.47, 0.86) was reduced. The cumulative incidence of pulmonary hypertension at 5 years was greater among those with late-age onset SSc (9%) compared to those with early-age onset SSc (2.7%; log-rank, p < 0.001).
These findings suggest that older patients with SSc are at greater risk for pulmonary hypertension, renal impairment, cardiac disease, and muscle weakness. Awareness of the distinct risk for specific organ manifestations in SSc, in particular pulmonary hypertension, should guide the care of patients with SSc whose disease begins after age 65.
尽管发生系统性硬化症（SSc）的老年患者（≥65 岁）可能表现出疾病的整个临床谱，但我们假设与早发性 SSc 患者相比，老年发病的 SSc 患者发生特定器官疾病表现的风险不同。
在 1990 年至 2009 年期间，共对 2300 例 SSc 患者进行了评估，并对来自大学硬皮病中心队列的患者进行了回顾性研究。对晚发性与早发性 SSc 患者的人口统计学特征、SSc 亚型、自身抗体状态、Medsger 严重程度评分、肺功能检查、超声心动图和右心导管检查结果进行比较。
总体而言，2084 例（91%）患者在 65 岁前发病，216 例（9%）患者≥65 岁。与早发性 SSc 患者相比，晚发性 SSc 患者的抗着丝点抗体比例显著更高（42%比 27%；p=0.001）。肺动脉高压（OR 1.76，95%CI 1.00，3.12）、肌肉无力（OR 1.85，95%CI 1.30，1.64）、肾功能不全（OR 2.83，95%CI 1.98，4.04）和心脏疾病（OR 2.69，95%CI 1.92，3.78）的风险较高，而手指缺血（OR 0.64，95%CI 0.47，0.86）的风险较低。5 年内，晚发性 SSc 患者的肺动脉高压累积发生率（9%）高于早发性 SSc 患者（2.7%；对数秩检验，p<0.001）。
这些发现表明，老年 SSc 患者发生肺动脉高压、肾功能不全、心脏疾病和肌肉无力的风险更高。了解 SSc 特定器官表现的不同风险，特别是肺动脉高压，应指导那些发病年龄大于 65 岁的 SSc 患者的治疗。
