Bussone Guillaume, Noël Laure-Hélène, Mouthon Luc
Inserm, université Paris Descartes, institut Cochin, rue Méchain, Paris, France.
Nephrol Ther. 2011 Jun;7(3):192-9. doi: 10.1016/j.nephro.2011.03.006. Epub 2011 Apr 27.
Scleroderma renal crisis is characterized by malignant hypertension and oligo-anuric acute renal failure. Scleroderma renal crisis occurs in 2 to 5% of patients with systemic sclerosis, particularly those with diffuse cutaneous systemic sclerosis in the first years of disease evolution. High-dose corticosteroid therapy (> 15 mg/d) is associated with an increased risk of scleroderma renal crisis. Patients present with prominent left heart failure and hypertensive encephalopathy. Renal failure can be associated with moderate proteinuria, without hematuria. Thrombotic microangiopathy is detected in 43% of the cases. Anti-RNA polymerase III antibodies are present in one third of patients with scleroderma renal crisis. In case of renal failure, iatrogenic or functional origin must be investigated, as well as crescentic glomerulonephritis associated with antineutrophil cytoplasm antibodies (ANCA) or thrombotic microangiopathy. Renal biopsy is not necessary to establish the diagnosis in typical forms of scleroderma renal crisis. However, it can help to evaluate the prognosis and it is recommended when clinical presentation of scleroderma renal crisis is unusual. The prognosis of scleroderma renal crisis dramatically improved with the use of angiotensin-converting enzyme (ACE) inhibitors. However, 5-year survival of patients who developed a scleroderma renal crisis is only 65%. The treatment relies on the early control of blood pressure with increasing doses of ACE inhibitors, in association with calcium channel blockers if necessary. In case of severe renal failure and/or hypertension, dialysis can help to quickly control the vascular overload and the blood pressure. Dialysis can be stopped in about half of cases. After 2 years on dialysis, eligible patients should be considered for renal transplantation. The prevention of scleroderma renal crisis lacks consensus. Corticosteroids and/or nephrotoxic drugs should be avoided in patients with diffuse cutaneous systemic sclerosis.
硬皮病肾危象的特征为恶性高血压和少尿性急性肾衰竭。硬皮病肾危象发生于2%至5%的系统性硬化症患者中，尤其是那些在疾病发展最初几年患有弥漫性皮肤系统性硬化症的患者。高剂量皮质类固醇治疗（>15毫克/天）会增加硬皮病肾危象的风险。患者表现为明显的左心衰竭和高血压脑病。肾衰竭可能伴有中度蛋白尿，无血尿。43%的病例检测到血栓性微血管病。三分之一的硬皮病肾危象患者存在抗RNA聚合酶III抗体。出现肾衰竭时，必须调查医源性或功能性病因，以及与抗中性粒细胞胞浆抗体（ANCA）相关的新月体性肾小球肾炎或血栓性微血管病。对于典型的硬皮病肾危象，肾活检并非确诊所必需。然而，它有助于评估预后，当硬皮病肾危象的临床表现不典型时建议进行肾活检。使用血管紧张素转换酶（ACE）抑制剂后，硬皮病肾危象的预后显著改善。然而，发生硬皮病肾危象的患者5年生存率仅为65%。治疗依赖于早期使用逐渐增加剂量的ACE抑制剂控制血压，必要时联合钙通道阻滞剂。出现严重肾衰竭和/或高血压时，透析有助于快速控制血管负荷和血压。约一半的病例可以停止透析。透析2年后，符合条件的患者应考虑进行肾移植。硬皮病肾危象的预防缺乏共识。弥漫性皮肤系统性硬化症患者应避免使用皮质类固醇和/或肾毒性药物。
