Department of Pulmonary Disease, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Rheumatol Int. 2012 Jun;32(6):1655-61. doi: 10.1007/s00296-011-1842-x. Epub 2011 Mar 5.
The objective of the current study was to determine survival and factors that affect survival in progressive systemic sclerosis (SSc) with pulmonary involvement. A total of 102 SSc patients with pulmonary involvement, diagnosed between 1994 and 2008, enrolled into the study. Pulmonary involvement was defined based on the presence of interstitial changes on high-resolution CT (HRCT). Demographical, clinical, radiological, and laboratory data of the patients were found from patient records and were used for the evaluation of survival. The mean age of the patients at diagnosis was 50 ± 12 years. In follow-up, 22 (21.5%) patients were deceased. The mean age of the patients at death was 55 ± 12 years. Seventy-eight percent of known causes of mortality were related to pulmonary complications. After any initial systemic manifestation of disease, the mean survival was 447 ± 27 months and 5, 10, 15, and 20 year survival rates were 99, 92, 83, and 72%, respectively. The mean survival after initial pulmonary manifestation was 269 ± 23 months and 5, 10 and 15 year survival rates were 91, 73 and 57%, respectively. Mean survival was 113 ± 5 months, and 5- and 10-year survival rates after the lung involvement detected with HRCT were 85 and 66%, respectively. Pulmonary artery hypertension, disease onset after the age of 40, and honeycombing on HRCT were associated with poor survival. Our data suggest that pulmonary involvement is the most important determinant factor for poor prognosis in patients with SSc. Therefore, echocardiography, pulmonary function tests, and HRCT should be performed in early stages of the disease for early diagnosis of pulmonary artery hypertension and lung involvement before irreversible vascular and interstitial changes exist.
本研究旨在确定有肺部受累的进行性系统性硬化症（SSc）患者的生存率及影响生存率的因素。共纳入 102 例在 1994 年至 2008 年间被诊断为有肺部受累的 SSc 患者。肺部受累的定义是基于高分辨率 CT（HRCT）存在间质改变。从患者病历中查找患者的人口统计学、临床、影像学和实验室数据，并用于评估生存率。诊断时患者的平均年龄为 50±12 岁。在随访期间，有 22 例（21.5%）患者死亡。死亡患者的平均年龄为 55±12 岁。已知的 78%死亡原因与肺部并发症相关。在任何疾病初始系统性表现后，平均生存时间为 447±27 个月，5、10、15 和 20 年生存率分别为 99%、92%、83%和 72%。在初始肺部表现后，平均生存时间为 269±23 个月，5、10 和 15 年生存率分别为 91%、73%和 57%。在 HRCT 检测到肺部受累后，平均生存时间为 113±5 个月，5 年和 10 年生存率分别为 85%和 66%。肺动脉高压、40 岁后发病和 HRCT 上的蜂巢样改变与不良预后相关。我们的数据表明，肺部受累是 SSc 患者预后不良的最重要决定因素。因此，在疾病早期应进行超声心动图、肺功能检查和 HRCT，以便在不可逆的血管和间质改变存在之前，早期诊断肺动脉高压和肺部受累。
