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结缔组织病所致弥漫性肺泡出血:以气道和呼吸管理为重点的综合综述

Connective Tissue Disorder-Induced Diffuse Alveolar Hemorrhage: A Comprehensive Review with an Emphasis on Airway and Respiratory Management.

作者信息

Mudgal Mayuri, Balaji Swetha, Gajendiran Ajeetha Priya, Subramanya Ananthraj, Murugan Shanjai Krishnan, Gondhi Venkatesh, Bhatnagar Aseem Rai, Gunasekaran Kulothungan

机构信息

Internal Medicine, Camden Clark Medical Center, Parkersburg, WV 26101, USA.

Internal Medicine, St. Mary's General Hospital, New York Medical College and St. Clare's Health, Passaic, NJ 07055, USA.

出版信息

Life (Basel). 2025 May 15;15(5):793. doi: 10.3390/life15050793.

Abstract

Diffuse alveolar hemorrhage (DAH), a catastrophic complication of connective tissue disorders (CTDs), manifests as rapid-onset hypoxemia, alveolar infiltrates, and progressive bleeding into the airways. While immune-mediated alveolar-endothelial injury primarily drives its pathophysiology, diagnosis is based on bronchoscopy and chest imaging. The clinical urgency lies in securing the compromised airway and stabilizing respiratory failure, a challenge increased by CTD-specific anatomical alterations such as cervical spine instability, cricoarytenoid arthritis, and subglottic stenosis. High-dose corticosteroids and immunosuppression are essential, while severe cases require extracorporeal membrane oxygenation or plasmapheresis. This comprehensive review introduces two novel approaches to address fundamental gaps in the management of CTD-induced DAH: a structured algorithm for a CTD-specific airway risk stratification tool, integrating anatomical screening and the application of lung ultrasounds (LUSs) for post-intubation CTD-induced DAH ventilation management. The need for a multidisciplinary team approach is also discussed. Despite aggressive care, mortality remains high (25-50%), underscoring the necessity for improved early recognition and intervention strategies for these high-risk patients.

摘要

弥漫性肺泡出血(DAH)是结缔组织病(CTD)的一种灾难性并发症,表现为迅速出现的低氧血症、肺泡浸润以及气道内进行性出血。虽然免疫介导的肺泡-内皮损伤是其主要病理生理学驱动因素,但诊断基于支气管镜检查和胸部影像学检查。临床紧迫性在于确保气道通畅并稳定呼吸衰竭,而CTD特有的解剖学改变(如颈椎不稳定、环杓关节炎和声门下狭窄)增加了这一挑战。大剂量皮质类固醇和免疫抑制治疗至关重要,而严重病例则需要体外膜肺氧合或血浆置换。这篇综述介绍了两种新方法,以填补CTD所致DAH管理中的基本空白:一种用于CTD特异性气道风险分层工具的结构化算法,整合解剖学筛查以及应用肺部超声(LUS)进行插管后CTD所致DAH的通气管理。还讨论了多学科团队方法的必要性。尽管积极治疗,但死亡率仍然很高(25%-50%),这突出了对这些高危患者改进早期识别和干预策略的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e119/12113093/6a3e6c5bf30b/life-15-00793-g001.jpg

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