BPF-GILD 研究:对英国鸽友的观察性队列研究

R. Allen, J. Wellens-Mensah, O. Leavy, S. Bourke, W. Henderson, H. Smith, E. Johnson, D. Marks, Y. Myat, C. Rafique, G. Parcesepe, T. Hernandez-Beeftink, B. Guillen-Guio, E. Cheng, C. Chan, G. Clark, S. Dickson, A. Pearson, M. Embley, P. Lynch, G. Boyd, B. Gooptu, Y. Ji, C. McSharry, L. Wain, M. Spears
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摘要

导言 超敏性肺炎(HP)是一种常见的间质性肺病(ILD),包括急性和纤维化(fHP)两种不同但相关的形式。过敏性肺炎通常被描述为一种由抗原暴露引发的疾病。然而,诱发因素的范围很广,约有 50% 的病例找不到病因,因此观察研究具有挑战性。英国鸽友ILD遗传学(BPF-GILD)研究旨在通过研究具有明确常见诱发因素接触史的人群来解决这一问题。方法 从 2019 年到 2023 年,在英国大型鸽友会议上招募参与者。每位参与者都进行了肺活量测定,与医生一起填写了一份标准化问卷,并提供了血液样本。我们在本手稿中介绍了我们的基线数据。结果 我们从四个鸽展中招募了 417 名受试者。受试者的年龄中位数为 63 岁,95% 为男性,94% 自称是白人。饲养鸽子数量的中位数为 80 只[范围为 4-800 只],鸽友每周在鸽舍中的时间为 14 小时[1-100 小时]。52%的参与者接触过职业性粉尘。49%的参与者称至少有一种呼吸道症状与鸽舍接触有关。14%的人有ILD病史,这些人与鸽舍相关的呼吸道症状较多、肺功能较差,而且与没有ILD病史的人相比(74%对57%),这些人似乎更有可能在遛鸽时戴上口罩。41%的参与者对ILD门诊中用来检测隐性结缔组织疾病的问题做出了积极的回答。讨论 我们这批特征明显的鸽友通常会出现急性高血压症状,并有可能增加罹患高血压的风险。利用储存的样本开展的后续工作将使我们能够确定遗传风险因素以及与发生 fHP 相关的途径。
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BPF-GILD study: an observational cohort study of UK pigeon fanciers
Introduction Hypersensitivity pneumonitis (HP), a common interstitial lung disease (ILD), comprises two distinct but related forms, acute and fibrotic (fHP). HP is classically described as a disease triggered by antigen exposure. However, a wide range of triggers are described and in ~50% of cases no cause is identified, rendering observational studies challenging. The British Pigeon Fanciers Genetics of ILD (BPF-GILD) study aims to address this by studying a population with a clear history of exposure to a common trigger. Methods Participants were recruited from 2019 to 2023 at large UK Pigeon Fancier meetings. Each participant performed spirometry, completed a standardised questionnaire with a doctor, and provided blood samples. We present our baseline data in this manuscript. Results 417 subjects were recruited from four shows. The median age of the cohort was 63 years, 95% were male and 94% self-reported white ethnicity. The median number of pigeons kept was 80 [range 4-800], with fanciers spending 14 hours per week [1-100] in their lofts. 52% of participants had occupational dust exposures. 49% of the cohort reported at least one respiratory symptom related to loft exposure. 14% had a history of ILD and these individuals had more loft-related respiratory symptoms, poorer lung function, and appeared more likely to wear a mask with their pigeons than those without (74% vs 57%). 41% of participants had positive responses to questions employed to detect occult connective tissue disease in ILD clinics. Discussion Our well characterised cohort of pigeon fanciers commonly experience acute HP symptoms and are likely to be at increased risk of fHP. Subsequent work using stored samples will enable us to determine genetic risk factors and pathways relevant to the development of fHP.
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