Lee P, Langevitz P, Alderdice C A, Aubrey M, Baer P A, Baron M, Buskila D, Dutz J P, Khostanteen I, Piper S
University of Toronto, Rheumatic Disease Unit, Wellesley Hospital, Ontario, Canada.
Q J Med. 1992 Feb;82(298):139-48.
Two hundred and thirty-seven patients with systemic sclerosis were followed prospectively in a scleroderma clinic. The overall 3, 6, and 9-year survival rates were 86, 76 and 61 per cent respectively. Renal, cardiac and pulmonary disease, and older age at enrollment were adverse prognostic factors associated with reduced survival. There were no significant differences in survival between males and females or in patients with restricted compared to those with diffuse skin thickening. Death from systemic sclerosis was most frequently due to pulmonary hypertension, with fewer than expected deaths from renal or cardiac causes. Twenty-eight per cent of deaths were due to causes unrelated to systemic sclerosis, most commonly cancer and ischaemic heart disease, and in older patients.
在一家硬皮病诊所对237例系统性硬化症患者进行了前瞻性随访。总体3年、6年和9年生存率分别为86%、76%和61%。肾脏、心脏和肺部疾病以及入组时年龄较大是与生存率降低相关的不良预后因素。男性和女性之间以及局限性皮肤增厚患者与弥漫性皮肤增厚患者之间的生存率无显著差异。系统性硬化症导致的死亡最常见的原因是肺动脉高压，因肾脏或心脏原因导致的死亡少于预期。28%的死亡是由与系统性硬化症无关的原因导致的，最常见的是癌症和缺血性心脏病，且多见于老年患者。
