肺-肾综合征，弥漫性肺出血和韦格纳肉芽肿病相关的快速进行性肾小球肾炎，临床病例和简短回顾

Diálisis y Trasplante Pub Date : 2013-07-01 DOI:10.1016/j.dialis.2012.12.003

Nasser Abdel Polanco Flores , María Virgilia Soto Abraham , Francisco Eugenio Rodríguez Castellanos

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摘要

我们报告一个41岁的墨西哥妇女的情况下，系统性小血管炎影响肾脏，缺乏免疫快速进行性肾小球肾炎和弥漫性肺出血。这种危及生命的疾病需要立即强化治疗，联合使用甲基强的松龙、环磷酰胺和血浆置换。患者表现出短暂的改善，随后病情逐渐恶化。治疗后肾活检结果显示活性高。b细胞淋巴细胞耗竭治疗(利妥昔单抗)开始，产生显著的临床和组织学改善，完全缓解。

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Síndrome pulmón-riñón, hemorragia pulmonar difusa y glomerulonefritis rápidamente progresiva asociada a granulomatosis de Wegener, caso clínico y revisión corta

We report the case of a 41-year-old Mexican woman with systemic small vessel vasculitis affecting the kidney with pauci-immune rapidly progressive glomerulonephritis and diffuse pulmonary hemorrhage. This life-threatening condition warrants immediate intensive treatment with combination therapy with methylprednisolone, cyclophosphamide, and plasma exchange. The patient showed a transient improvement, with subsequent progressive deterioration. The results of renal biopsy showed high activity after treatment. B-cell lymphocyte depletion therapy (rituximab) was started, producing substantial clinical and histologic improvement, with complete remission.

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Diálisis y Trasplante

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