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[弥漫性肺泡出血——风湿病学中急性、危及生命的情况]

[Diffuse alveolar hemorrhage - acute, life-threatening situation in rheumatology].

作者信息

Bradna Petr, Maňák Jan, Soukup Tomáš, Kodeda Michal, Drahošová Marcela, Tomš Jan

出版信息

Vnitr Lek. 2018 Spring;64(2):169-172.

PMID:29595282
Abstract

Diffuse alveolar hemorrhage (DAH) is a life-threatening acute manifestation of systemic diseases, the most commonly of systemic vasculitis. Clinically DAH manifests by a rapidly progressive respiratory and renal failure. The decisive for diagnose is immediate bronchoscopic examination with the bronchoalveolar lavage examination. CT mostly show bilateral pulmonary infiltrates, in blood picture rapidly come to anemia. In the majority of patients it can be found positive ANCA antibodies. DAH should be suspected in the case of acute respiratory failure also in patients without history of systemic disease. On the set of 33 patients with acute DAH episode, we demonstrate the importance of rapid diagnosis and aggressive therapy. In a third of our patients was DAH the first manifestation of systemic disease. Immunomodulatory treatment must be initiated immediately after diagnose. Hospital mortality in our group was 27 %, although 42 % of the patients were required ventilation support and one-third of patients had acute renal failure. After handling of acute episode of DAH is the prognosis quoad vitam promising.Key words: bronchoalveolar fluid - diffuse alveolar haemorrhage - granulomatosis with polyangiitis - intensive care in rheumatology - vasculitis.

摘要

弥漫性肺泡出血(DAH)是系统性疾病的一种危及生命的急性表现,最常见于系统性血管炎。临床上,DAH表现为快速进展的呼吸和肾衰竭。诊断的关键是立即进行支气管镜检查及支气管肺泡灌洗检查。CT大多显示双侧肺部浸润,血常规迅速出现贫血。大多数患者可检测到抗中性粒细胞胞浆抗体(ANCA)阳性。即使没有系统性疾病史的患者,出现急性呼吸衰竭时也应怀疑DAH。在33例急性DAH发作的患者中,我们证明了快速诊断和积极治疗的重要性。在我们三分之一的患者中,DAH是系统性疾病的首发表现。诊断后必须立即开始免疫调节治疗。尽管42%的患者需要通气支持,三分之一的患者有急性肾衰竭,但我们组的医院死亡率为27%。处理DAH急性发作后,患者的生命预后良好。关键词:支气管肺泡灌洗液 - 弥漫性肺泡出血 - 肉芽肿性多血管炎 - 风湿病重症监护 - 血管炎

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