Scleroderma renal crisis: patient characteristics and long-term outcomes
Scleroderma renal crisis: patient characteristics and long-term outcomes
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DOI：
10.1093/qjmed/hcm052
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发表时间：
2007-08-01
影响因子：
13.3
通讯作者：
Denton, C. P.
中科院分区：
文献类型：
--
作者：
Penn, H.;Howie, A. J.;Denton, C. P.
背景：硬皮病肾危象（SRC）是系统性硬化症的重要并发症，可引起急性肾功能衰竭，通常伴有高血压。目的：回顾SRC的临床和病理特征，并将其与肾脏预后和死亡率联系起来。设计：回顾性病例系列。方法：从1990年到2005年，我们在同一中心鉴定了110例SRC。结果：随访中硬皮病患者SRC发生率为5%。病例以女性为主（81%），伴弥漫性皮肤病（78%）。在59%的检测病例中发现RNA聚合酶抗体。几乎所有（108/110）患者接受了ACE抑制剂（ACEls）治疗。36%的患者不需要透析，23%的患者需要临时透析（长达3年），41%的患者需要永久透析。未进行透析的患者在SRC后估计肾小球滤过率有所改善（3年内平均变化+23 ml/min）。肾脏预后差与就诊时血压较低有关，且需要透析的患者年龄较大。类固醇使用、微血管病溶血性贫血和抗体谱与肾脏预后无关。在可用于临床相关性的58例肾脏活检中，动脉粘膜内膜增厚和小动脉纤维蛋白样坏死的急性变化与较差的肾脏预后相关。死亡率很高（5年生存率为59%），男性死亡率更高。讨论：尽管acel在治疗SRC方面有疗效，但其不良的长期结果值得对这种系统性硬化症的毁灭性并发症进行额外治疗的评估。
Background: Scleroderma renal crisis (SRC) is an important complication of systemic sclerosis, causing acute renal failure, and usually hypertension. Aims: To review the clinical and pathological features of SRC, and correlate them with renal outcomes and mortality. Design: Retrospective case series. Methods: We identified 110 cases of SRC managed at a single centre between 1990 and 2005. Results: SRC occurred in 5% of scleroderma cases under follow-up. Cases were predominantly female (81%), with diffuse cutaneous disease (78%). RNA polymerase antibodies were found in 59% of cases tested. Almost all (108/110) received treatment with ACE inhibitors (ACEls). Dialysis was not required in 36%, was required temporarily (for up to 3 years) in 23%, was required permanently in 41%. Patients not on dialysis showed improvement in estimated glomerular filtration rate after SRC (mean change +23 ml/min over 3 years). Poor renal outcome was associated with lower blood pressure at presentation, and with higher age in those requiring dialysis. Steroid use, microangiopathic haemolytic anaemia, and antibody profile were not related to renal outcome. In the 58 renal biopsies available for clinical correlation, acute changes of mucoid intimal thickening in arteries and fibrinoid necrosis in arterioles were associated with a poorer renal outcome. Mortality was high (59% survival at 5 years), and was higher in men. Discussion: Despite the efficacy of ACEls in managing SRC, the poor long-term outcome warrants evaluation for additional treatments for this devastating complication of systemic sclerosis.