Division of Rheumatology, University of Pennsylvania, 8th Floor Penn Tower; One Convention Blvd, Philadelphia, PA 19104, USA.
Rheumatol Int. 2012 Dec;32(12):3841-4. doi: 10.1007/s00296-011-2301-4. Epub 2011 Dec 23.
To compare the characteristics of patients with systemic sclerosis who died within 2 years of diagnosis to those who died after 2 years of diagnosis. A retrospective chart review of all medical records of deceased systemic sclerosis (SSc) patients who had been followed at our institution from 1985 to 2007 was performed. We identified 87 deceased SSc patients within this period. From the 87 deceased individuals, 20 had died within 2 years after they were diagnosed, and 67 had died after 2 years of their diagnosis. Patients who died within 2 years of diagnosis were more likely to be anticentromere antibody negative when compared to the patients who died after 2 years (17/20 vs. 48/67, P = 0.03). The time from the first appearance of non-Raynaud's symptoms to diagnosis was significantly shorter in the group who died within 2 years than in the group who died after 2 years of diagnosis (11.8 ± 10.3 vs. 60.7 ± 64.9 months, P = 0.002). According to the Medsger severity score, there was more severe muscle (0.82 ± 1.13 vs. 1.8 ± 1.28, P = 0.0014) and heart (0.86 ± 1.37 vs. 2.1 ± 1.71, P = 0.0013) involvement at the initial evaluation in patients who died before 2 years of diagnosis when compared to the group of patients who died after 2 years of diagnosis. The time from the first symptoms to treatment initiation was significantly shorter in patients who died early (9.43 ± 6.3 vs. 38.3 ± 54.4 months, P = 0.05). The interval between treatment initiation and death was also significantly shorter (15.1 ± 9.48 vs. 60.7 ± 49.7 months, P = 0.001), reflecting greater severity of disease. Patients who died within the first 2 years of SSc diagnosis were typically anticentromere negative and had significant muscle and cardiac involvement. The time from the first appearance of non-Raynaud phenomenon symptoms to death was much shorter in the patients who died within 2 years of diagnosis, suggesting a very fulminant form of systemic sclerosis.
为了比较系统性硬化症（SSc）患者在诊断后 2 年内死亡与 2 年后死亡的特征。对 1985 年至 2007 年在本机构就诊的所有已故 SSc 患者的病历进行了回顾性图表分析。在此期间，我们共发现 87 名已故 SSc 患者。在这 87 名死亡个体中，有 20 人在诊断后 2 年内死亡，有 67 人在诊断后 2 年后死亡。与诊断后 2 年后死亡的患者相比，诊断后 2 年内死亡的患者更可能为抗着丝点抗体阴性（17/20 例 vs. 48/67 例，P = 0.03）。在诊断后 2 年内死亡的患者中，从首发非雷诺现象症状到诊断的时间明显短于诊断后 2 年后死亡的患者（11.8 ± 10.3 个月 vs. 60.7 ± 64.9 个月，P = 0.002）。根据 Medsger 严重程度评分，在诊断后 2 年内死亡的患者中，肌肉（0.82 ± 1.13 分 vs. 1.8 ± 1.28 分，P = 0.0014）和心脏（0.86 ± 1.37 分 vs. 2.1 ± 1.71 分，P = 0.0013）的初始评估受累更为严重。与诊断后 2 年后死亡的患者相比，早期死亡患者（9.43 ± 6.3 个月 vs. 38.3 ± 54.4 个月，P = 0.05）从首发症状到开始治疗的时间明显缩短。早期治疗开始与死亡之间的间隔也明显缩短（15.1 ± 9.48 个月 vs. 60.7 ± 49.7 个月，P = 0.001），这反映了疾病的严重程度更高。在诊断后 2 年内死亡的 SSc 患者通常为抗着丝点抗体阴性，且有明显的肌肉和心脏受累。在诊断后 2 年内死亡的患者中，从首发非雷诺现象症状到死亡的时间明显缩短，提示存在非常暴发性的系统性硬化症。
