Long-term outcomes of scleroderma renal crisis
Long-term outcomes of scleroderma renal crisis
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DOI：
10.7326/0003-4819-133-8-200010170-00010
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发表时间：
2000-10-17
影响因子：
39.2
通讯作者：
Medsger, TA
中科院分区：
文献类型：
--
作者：
Steen, VD;Medsger, TA
背景：虽然硬皮病肾危象（系统性硬化症的一种并发症）可以用血管紧张素转换酶 (ACE) 抑制剂治疗，但其长期结局尚不清楚。目的：确定系统性硬化症和硬皮病肾危象患者的结局、自然病程和危险因素。设计：前瞻性观察队列研究。背景：硬皮病专业大学项目。患者：145 名接受过硬皮病肾危象治疗的硬皮病肾危象患者。 ACE抑制剂和662名没有肾危象的硬皮病患者。测量：在肾危象患者中，研究的四种结果是不透析、临时透析、永久透析和早期死亡。比较人口统计、临床和实验室数据，以确定特定结果的风险因素。随访时间为5至10年。结果：61%的肾危象患者预后良好（55例未接受透析，34例接受临时透析）；其中只有 4 例 (4%) 进展为慢性肾功能衰竭和永久性透析。超过一半最初需要透析的患者可以在 3 至 18 个月后停止透析。良好结果组患者的生存率与未出现肾危象的弥漫性硬皮病患者相似。一些患者 (39%) 预后不良（永久透析或过早死亡）。结论：当使用 ACE 抑制剂积极控制高血压时，可以有效控制肾危象。即使开始透析后，患者也应继续服用 ACE 抑制剂，以期停止透析。
Background: Although scleroderma renal crisis, a complication of systemic sclerosis, can be treated with angiotensin-converting enzyme (ACE) inhibitors, its long-term outcomes are not known.Objective: To determine outcomes, natural history, and risk factors in patients with systemic sclerosis and scleroderma renal crisis.Design: Prospective observational cohort study.Setting: University program specializing in scleroderma.Patients: 145 patients with scleroderma renal crisis who received ACE inhibitors and 662 patients with scleroderma who did not have renal crisis.Measurements: Among patients with renal crisis, the four outcomes studied were no dialysis, temporary dialysis, permanent dialysis, and early death. Demographic, clinical, and laboratory data were compared to identify risk factors for specific outcomes. Follow-up was 5 to 10 years.Results: 61% of patients with renal crisis had good outcomes (55 received no dialysis, and 34 received temporary dialysis); only 4 of these (4%) progressed to chronic renal failure and permanent dialysis. More than half of the patients who Initially required dialysis could discontinue it 3 to 18 months later. Survival of patients in the good outcome group was similar to that of patients with diffuse scleroderma who did not have renal crisis. Some patients (39%) had bad outcomes (permanent dialysis or early death).Conclusions: Renal crisis can be effectively managed when hypertension is aggressively controlled with ACE inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hopes of discontinuing dialysis.