Changes in causes of death in systemic sclerosis, 1972-2002
Changes in causes of death in systemic sclerosis, 1972-2002
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DOI：
10.1136/ard.2006.066068
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发表时间：
2007-07-01
影响因子：
27.4
通讯作者：
Medsger, Thomas A.
中科院分区：
文献类型：
--
作者：
Steen, Virginia D.;Medsger, Thomas A.
背景：自从肾危象治疗成为可能后，硬皮病的生存率发生了变化。目的：为了记录的变化，在生存和器官系统的死亡原因在系统性硬化症（SSc）在过去的25年中的患者从一个单一的医疗center.Methods：对比患者在匹兹堡大学，匹兹堡，宾夕法尼亚州，美国1972年1月1日至1996年12月31日进行了研究。生存率在1972年至1997年的五个5年时间段内确定。死亡原因包括硬皮病（硬皮病肾危象、肺动脉高压、肺纤维化（PF）、胃肠道（GI）、心脏和多器官衰竭）和非硬皮病相关（癌症、动脉粥样硬化性心血管或脑血管疾病、感染、猝死、其他和未知）原因。在每个时间间隔内，10年生存率从54%稳步提高到66%。与1972 - 81年相比，1982 - 91年患者的生存率显著提高（p < 0.001），即使排除了肾危象患者（p < 0.005）。在30年的时间里，因肾危象而死亡的频率显着下降，硬皮病相关死亡的比例从42%下降到6%（p < 0.001），而死于PF的硬皮病患者比例从6%增加到33%（p < 0.001）。在此期间，肺动脉高压的频率（与PF无关）也显著增加（p，0.05）。硬皮病胃肠道和心脏相关的死亡没有变化，任何非硬皮病相关的原因也没有变化，尽管硬皮病患者在过去15年中死于硬皮病相关问题的可能性较低。过去30年来硬皮病相关死亡模式的变化表明，（肺动脉高压和PF）是当今硬皮病相关死亡的主要原因。重要的是，积极的搜索继续开发更好的治疗这些严重的肺部并发症的SSc。
Background: Survival of scleroderma has changed since the renal crisis treatment has become possible. Aims: To document the changes in survival and organ system causes of mortality in systemic sclerosis ( SSc) over the past 25 years in patients from a single medical centre.Methods: Consecutive patients evaluated at the University of Pittsburgh, Pittsburgh, Pennsylvania, USA between 1 January 1972 and 31 December 1996 were studied. Survival was determined in five 5- year time periods between 1972 and 1997. Causes of death included scleroderma- related ( scleroderma renal crisis, pulmonary arterial hypertension, pulmonary fibrosis ( PF), gastrointestinal ( GI), heart and multiorgan failure) and non- scleroderma- related ( cancer, atherosclerotic cardiovascular or cerebrovascular disease, infection, sudden death, other and unknown) causes.Results: The 10- year survival improved steadily from 54% to 66% during each of the time intervals. There was a significant improvement in survival for patients during 1982 - 91 compared with those during 1972 - 81 ( p < 0.001), even when patients with renal crisis were excluded ( p < 0.005). The frequency of deaths due to renal crisis significantly decreased over the 30- year time period, from 42% to 6% of scleroderma- related deaths ( p < 0.001), whereas the proportion of patients with scleroderma who died of PF increased from 6% to 33% ( p < 0.001). The frequency of pulmonary hypertension, independent of PF, also significantly increased during this time period ( p, 0.05). There were no changes in scleroderma GI- and heart- related deaths, nor in any of the non- scleroderma- related causes, although patients with scleroderma were less likely to die from scleroderma- related problems in the past 15 years.Conclusion: The change in the pattern of scleroderma- related deaths over the past 30 years demonstrates that the lung ( both pulmonary hypertension and PF) is the primary cause of scleroderma- related deaths today. It is important that aggressive searches continue to develop better therapies for these severe pulmonary complications of SSc.