Denton C P, Lapadula G, Mouthon L, Müller-Ladner U
Centre for Rheumatology, Royal Free Hospital, Rheumatology Unit, London, UK.
Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii32-5. doi: 10.1093/rheumatology/ken483.
Scleroderma renal crisis (SRC) occurs in 5-10% of SSc patients, who may present with an abrupt onset of hypertension, acute renal failure, headaches, fevers, malaise, hypertensive retinopathy, encephalopathy and pulmonary oedema. Patients at greatest risk of developing SRC are those with diffuse cutaneous or rapidly progressive forms of SSc, and treatment with a recently commenced high dose of corticosteroid. Laboratory tests may demonstrate hypercreatinaemia, microangiopathic haemolytic anaemia (MAHA), thrombocytopaenia and hyperreninaemia. Renal crisis is also linked to a positive ANA speckled pattern, antibodies to RNA polymerase I and II, and an absence of anti-centromere antibodies. Early, aggressive treatment with angiotensin-converting enzyme inhibitors has improved prognosis in SRC, although 40% of the patients may require dialysis, and mortality at 5 yrs is 30-40%. Median time to recovery is 1 yr, and typically occurs within 3 yrs. Prognosis is worse for males, but may not be related to corticosteroid use, presence of MAHA or severity of renal pathology. Modification of endothelin over-activity, which is implicated in the pathogenesis of SRC, may offer a future therapeutic approach.
硬皮病肾危象（SRC）发生于5% - 10%的系统性硬化症（SSc）患者中，这些患者可能会突然出现高血压、急性肾衰竭、头痛、发热、不适、高血压性视网膜病变、脑病和肺水肿。发生SRC风险最高的患者是那些患有弥漫性皮肤型或快速进展型SSc且近期开始大剂量使用皮质类固醇治疗的患者。实验室检查可能显示血肌酐升高、微血管病性溶血性贫血（MAHA）、血小板减少和高肾素血症。肾危象还与抗核抗体斑点型阳性、抗RNA聚合酶I和II抗体以及无抗着丝点抗体有关。尽管40%的患者可能需要透析，且5年死亡率为30% - 40%，但早期积极使用血管紧张素转换酶抑制剂治疗已改善了SRC的预后。恢复的中位时间为1年，通常在3年内发生。男性的预后较差，但可能与使用皮质类固醇、MAHA的存在或肾脏病理的严重程度无关。内皮素过度活跃在SRC的发病机制中起作用，对其进行调节可能会提供一种未来的治疗方法。
