Sporadic Obliterative Bronchiolitis: Case Series and Systematic Review of the Literature.
Sporadic Obliterative Bronchiolitis: Case Series and Systematic Review of the Literature.
复制标题
散发性闭塞性细支气管炎：病例系列和文献系统回顾。
DOI：
10.1016/j.mayocpiqo.2018.10.003
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发表时间：
2019
期刊：
影响因子：
--
通讯作者：
Mehrad,Borna
中科院分区：
文献类型：
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作者：
Callahan,SeanJ;Vranic,Andrew;Flors,Lucia;Hanley,Michael;Stoler,MarkH;Mehrad,Borna
目的描述闭塞性细支气管炎（OB）的临床特征和结果诊断与移植或点源暴露吸入toxics.Patients和MethodsWe收集所有确诊的OB在我们的机构和分析他们的人口统计学特征，治疗和结果定义的肺功能检查（PFT）和无移植死亡率。研究期间为2007年7月至2017年8月。组织学诊断由病理学家确认，高分辨率胸部计算机断层扫描（CT）扫描由胸部放射科医生进行审查和评分。我们还进行了系统的文献回顾散发OB series.ResultsWe确定了19例确诊病例在我们的机构和9篇文献中包含104例患者。在我们的系列和文献中，患者不成比例地为中年白人女性。42%的患者为特发性疾病，分别有31%和15%的患者与结缔组织疾病和吸入性暴露有关。胸部CT显示所有患者均存在呼气相空气潴留。分别有77%、46%和22%的患者接受皮质类固醇、类固醇保留剂和大环内酯类药物治疗。在我们1703天（范围11-3206天）的中位随访中，PFT没有显著变化。在合并的所有系列中，全因死亡率为82/1000患者-年（95% CI，65-102）。14例死亡，3例死亡是由于呼吸衰竭和5可能与并发症的免疫抑制therapeutic.Conclusionsporadic OB是一种罕见的疾病，是均匀相关的高分辨率胸部CT空气滞留。如有可能，应通过手术活检确诊。这种疾病通常不是渐进的。
ObjectiveTo describe the clinical characteristics and outcomes of patients diagnosed with obliterative bronchiolitis (OB) not associated with transplantation or point-source exposures to inhaled toxins.Patients and MethodsWe compiled all confirmed diagnoses of OB at our institution and analyzed their demographic characteristics, treatments, and outcomes as defined by pulmonary function tests (PFTs) and transplant-free mortality. The study period ranged from July 2007 to August 2017. Histological diagnosis was confirmed by a pathologist, and high-resolution chest computed tomography (CT) scans were reviewed and scored by chest radiologists. We also performed a systematic literature review of sporadic OB series.ResultsWe identified 19 confirmed cases at our institution and 9 publications in the literature containing 104 patients. In both our series and the literature, patients were disproportionately middle-aged Caucasian women. The disease was idiopathic in 42% and was associated with connective tissue diseases and inhalational exposures in 31% and 15%, respectively. Chest CT showed expiratory air trapping in all patients. Patients were treated with corticosteroids, steroid-sparing agents, and macrolides in 77%, 46%, and 22%, respectively. Over a median follow-up in our series of 1703 days (range, 11-3206 days), PFTs did not change significantly. In all series combined, mortality incidence from any cause was 82/1000 patient-years (95% CI, 65-102). Of 14 patients who died, 3 deaths were due to respiratory failure and 5 were potentially related to complications of immunosuppressive therapy.ConclusionSporadic OB is a rare disease that is uniformly associated with air trapping on high-resolution chest CT. The diagnosis should be established with surgical biopsy if possible. The illness is not typically progressive.