Scleroderma renal crisis
Scleroderma renal crisis
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DOI：
10.1016/s0889-857x(03)00016-4
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发表时间：
2003-05-01
影响因子：
2.3
通讯作者：
Steen, VD
中科院分区：
文献类型：
--
作者：
Steen, VD
肾脏危象发生在患有系统性硬化症的患者中，这些患者在疾病早期迅速进行性弥漫性皮肤增厚。硬皮病肾危象以恶性高血压、高肾素血症、氮质血症、微血管病理性溶血性贫血和肾功能衰竭为特征。这种并发症几乎都是致命的，但在大多数情况下，现在可以用血管紧张素转换酶(ACE)抑制剂成功地治疗它。这种疗法提高了存活率，减少了透析的需要，并经常允许在6到18个月后停止透析。及时诊断和及早积极地开始使用血管紧张素转换酶抑制剂治疗将产生最理想的结果。
Renal crisis occurs in patients who have systemic sclerosis with rapidly progressive diffuse cutaneous thickening early in their disease. Scleroderma renal crisis is characterized by malignant hypertension, hyperreninemia, azotemia, microangiopathic hemolytic anemia, and renal failure. This complication was almost uniformly fatal, but in most cases it can now be successfully treated with angiotensin-converting enzyme (ACE) inhibitors. This therapy has improved survival, reduced the requirement for dialysis, and often allowed for the discontinuation of dialysis 6 to 18 months later. Prompt diagnosis and early, aggressive initiation of therapy with ACE inhibitors will result in the most optimal outcome.