Febres-Aldana Christopher A, Oneto Sabrina, Csete Marc, Vincentelli Cristina
Arkadi M. Rywlin M.D. Department of Pathology and Laboratory Medicine, Mount Sinai Medical Center, Miami Beach, FL, USA.
Department of Critical Care, Mount Sinai Medical Center, Miami Beach, FL, USA.
Respir Med Case Rep. 2018 Dec 21;26:136-141. doi: 10.1016/j.rmcr.2018.12.013. eCollection 2019.
Non-fully reversible airway obstruction in fatal asthma is often seen in association with profound structural changes of the bronchial wall, termed airway remodeling. Evidence suggests that heavy precipitation events can trigger epidemics of severe asthma. We present a case of fatal asthma in a young woman with no prior near-fatal exacerbations and postulate that the patient's extensive airway remodeling and puerperal state (susceptibility factors), in combination with a massive allergen challenge during a hurricane landfall (triggering factor), played a central role in her death. The autopsy revealed diffuse obstruction of proximal and distal bronchi by mucous plugs together with transmural chronic inflammation, tissue eosinophilia, extensive goblet cell hyperplasia with MUC-5 expression and airway smooth muscle (ASM) thickening. The observed distribution of airway remodeling was heterogeneous with sparing of the lingula, which exhibited hyperinflation and expansion of perivascular spaces indicative of dissecting air. The massive stagnation of mucus and significant inter-airway structural heterogeneity created an anatomical substrate for unequal airflow distribution facilitating the development of barotrauma. Although not considered conventional risk factors for fatal asthma, we believe that in this case, the patient's puerperal state in conjunction with an extreme environmental event dispersing aeroallergens were major contributors to the development of a fatal asthma attack. Our autopsy findings suggest that effective strategies to evacuate stagnated mucus and induce relaxation of thickened ASM are crucial in the management of life-threatening asthma exacerbations.
致死性哮喘中不完全可逆的气道阻塞常与支气管壁的显著结构改变相关,即气道重塑。有证据表明,强降水事件可引发严重哮喘的流行。我们报告一例年轻女性致死性哮喘病例,该患者既往无濒死加重史,并推测患者广泛的气道重塑和产褥期状态(易感因素),与飓风登陆期间大量过敏原激发(触发因素)共同作用,在其死亡中起核心作用。尸检显示,近端和远端支气管被黏液栓弥漫性阻塞,伴有透壁性慢性炎症、组织嗜酸性粒细胞增多、广泛的杯状细胞增生伴MUC - 5表达以及气道平滑肌(ASM)增厚。观察到的气道重塑分布不均,舌叶未受累,表现为过度充气和血管周围间隙扩大,提示存在间质性肺气肿。大量黏液潴留和气道间显著的结构异质性为气流分布不均创造了解剖学基础,促进了气压伤的发生。尽管不被视为致死性哮喘的传统危险因素,但我们认为,在该病例中,患者的产褥期状态与极端环境事件导致气传过敏原扩散是致死性哮喘发作的主要促成因素。我们的尸检结果表明,有效清除潴留黏液和诱导增厚的ASM松弛的策略对于危及生命的哮喘加重的管理至关重要。