Management of idiopathic crescentic and diffuse proliferative glomerulonephritis: Evidence-based recommendations
Management of idiopathic crescentic and diffuse proliferative glomerulonephritis: Evidence-based recommendations
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DOI：
10.1046/j.1523-1755.1999.07005.x
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发表时间：
1999-06-01
影响因子：
19.6
通讯作者：
Jindal, KK
中科院分区：
文献类型：
--
作者：
Jindal, KK
特发性新月体肾小球肾炎（GN）通常表现为肾功能的快速丧失和病理学显示广泛的新月体形成。该疾病是由不同的免疫发病机制引起的，缺乏免疫，通常是抗神经细胞胞质抗体（ANCA）阳性的微血管炎，抗肾小球基底膜（GBM）抗体疾病，和免疫复合物的形成。历史回顾显示，即使在口服类固醇和细胞毒性药物治疗后，肾脏预后也很差。在过去十年中，预后有所改善。在这篇文章中，提出了基于证据的管理建议。由于终末期肾病（ESRD）的高风险，尽管支持证据不足，但仍建议早期积极治疗。抗GEM抗体诱导的新月体GN应尽早开始治疗，治疗应包括甲基强的松龙冲击、为期2周的血浆置换和2个月的皮质类固醇和环磷酰胺治疗（B级和C级）。对缺乏免疫力的新月体肾炎的治疗应该是甲基强的松龙冲击，然后口服皮质类固醇和环磷酰胺6至12个月（B级）。复发可以类似地管理（B级），沿着适当的支持治疗。在发展为ESRD的患者中，可以进行成功的移植。弥漫性毛细血管内增生性肾小球肾炎是典型的感染后病变。当没有新月体形成时，通常预后良好。持续性蛋白尿、高血压和肾功能损害的成人患者需要仔细随访和管理，以改善进行性血流动力学损伤。
Idiopathic crescentic glomerulonephritis (GN) often presents with a rapid loss of renal function and pathology showing extensive crescent formation. The disease is caused by different immunopathogenetic mechanisms, pauci-immune, often antineutrophil cytoplasmic antibody (ANCA)-positive microvasculitis, antiglomerular basement membrane (GBM) antibody disease, and immune complex formation. Historical reviews reveal poor renal prognosis, even after treatment with oral steroids and cytotoxic drugs. Prognosis has improved in the last decade. In this article, evidence-based recommendations for management are presented. Because of the high risk of end-stage renal disease (ESRD), early aggressive therapy is recommended, despite weak supporting evidence. Treatment for anti-GEM antibody-induced crescentic GN should be initiated early and should include pulse methylprednisolone, a two-week course of plasmapheresis and two months of treatment with corticosteroids and cyclophosphamide (grade B and C). Treatment for pauci-immune crescentic GN should be pulse methylprednisolone, followed by oral corticosteroids and cyclophosphamide for 6 to 12 months (grade B). Recurrences can be managed similarly (grade B), along with appropriate supportive therapy. In patients who develop ESRD, successful transplantation can be performed. Diffuse endocapillary proliferative GN is classically postinfectious. It generally has a good prognosis when no crescent formation occurs. Adult patients with persistent proteinuria, hypertension, and renal function impairment need careful follow-up and management to modify progressive hemodynamic injury.