Bench-to-bedside review: pulmonary-renal syndromes--an update for the intensivist.

Bench-to-bedside review: pulmonary-renal syndromes--an update for the intensivist.
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基准对床的评论:肺肾脏综合征 - 强化主义者的更新。

DOI:
10.1186/cc5778
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发表时间:
2007
期刊:
影响因子:
15.1
通讯作者:
Roussos, Charis
Roussos, Charis
中科院分区:
医学1区
文献类型:
--
作者:
Papiris, Spyros A.;Manali, Effrosyni D.;Kalomenidis, Ioannis;Kapotsis, Giorgios E.;Karakatsani, Anna;Roussos, Charis

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术语肺肾综合征是指弥漫性肺泡出血和快速进行性肾小球肾炎的组合。多种机制,如涉及抗肾小球基底膜抗体,抗神经元胞质抗体或免疫复合物和血栓性微血管病的发病机制,涉及这种综合征。潜在的肺部病理学是小血管炎,累及小动脉、小静脉,经常累及肺泡毛细血管。潜在的肾脏病理学是一种局灶性增生性肾小球肾炎。免疫荧光有助于区分抗肾小球基底膜疾病(IgG的线性沉积)、狼疮和感染后肾小球肾炎(免疫球蛋白和补体的颗粒沉积)以及坏死性血管炎(少免疫性肾小球肾炎)。患者可能出现严重呼吸和/或肾衰竭,需要入住重症监护室。由于该综合征的特点是如果不及时治疗,则会发生暴发性病程,因此早期诊断、排除感染、密切监测患者和及时开始治疗对患者的预后至关重要。治疗包括高剂量的皮质类固醇和细胞毒性药物,在某些情况下结合血浆置换。肾移植是治疗终末期肾病的唯一选择。较新的免疫调节剂,如那些导致TNF阻断,B细胞耗竭和霉酚酸酯可用于难治性疾病的患者。
The term Pulmonary–renal syndrome refers to the combination of diffuse alveolar haemorrhage and rapidly progressive glomerulonephritis. A variety of mechanisms such as those involving antiglomerular basement membrane antibodies, antineutrophil cytoplasm antibodies or immunocomplexes and thrombotic microangiopathy are implicated in the pathogenesis of this syndrome. The underlying pulmonary pathology is small-vessel vasculitis involving arterioles, venules and, frequently, alveolar capillaries. The underlying renal pathology is a form of focal proliferative glomerulonephritis. Immunofluorescence helps to distinguish between antiglomerular basement membrane disease (linear deposition of IgG), lupus and postinfectious glomerulonephritis (granular deposition of immunoglobulin and complement) and necrotizing vasculitis (pauci-immune glomerulonephritis). Patients may present with severe respiratory and/or renal failure and require admission to the intensive care unit. Since the syndrome is characterized by a fulminant course if left untreated, early diagnosis, exclusion of infection, close monitoring of the patient and timely initiation of treatment are crucial for the patient's outcome. Treatment consists of corticosteroids in high doses, and cytotoxic agents coupled with plasma exchange in certain cases. Renal transplantation is the only alternative in end-stage renal disease. Newer immunomodulatory agents such as those causing TNF blockade, B-cell depletion and mycophenolate mofetil could be used in patients with refractory disease.
DOI: 10.1097/01.asn.0000114554.67106.28
发表时间: 2004-03-01
影响因子: 13.6
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