Penn State Health Allergy, Asthma, and Immunology, Hershey, Pa.
Family Allergy and Asthma, Louisville, Ky.
J Allergy Clin Immunol Pract. 2023 Aug;11(8):2348-2354. doi: 10.1016/j.jaip.2023.04.027. Epub 2023 May 3.
Alpha-1 antitrypsin deficiency (AATD) is a genetic disorder that manifests as lung and/or liver disease. Because symptoms of AATD overlap with those of common pulmonary and hepatic conditions, AATD is often misdiagnosed, which has resulted in substantial underdiagnosis of AATD worldwide. Although screening patients for AATD is recommended, the lack of procedures to facilitate testing remains a barrier to accurate diagnosis of AATD. Delays in AATD diagnosis can worsen outcomes for patients by postponing appropriate disease-modifying treatments. Patients with AATD-related lung disease experience symptoms similar to other obstructive lung disorders and are often misdiagnosed for years. In addition to existing screening guidelines, we recommend that screening for AATD become a standard part of allergists' workups of patients with asthma and fixed obstructive disease, chronic obstructive pulmonary disease, bronchiectasis without known origin, and patients under consideration for treatment with biologics. This Rostrum article reviews screening and diagnostic tests available in the United States and emphasizes evidence-based strategies to increase testing frequency and improve AATD detection rates. We underscore the pivotal role of allergists in managing care for patients with AATD. Finally, we urge health care providers to be aware of potentially poor clinical outcomes among patients with AATD during the coronavirus disease 2019 pandemic.
α-1 抗胰蛋白酶缺乏症(AATD)是一种遗传疾病,表现为肺部和/或肝脏疾病。由于 AATD 的症状与常见的肺部和肝脏疾病重叠,因此 AATD 经常被误诊,这导致了全球范围内 AATD 的大量漏诊。尽管建议对 AATD 患者进行筛查,但缺乏便于检测的程序仍然是准确诊断 AATD 的障碍。AATD 诊断的延迟会通过推迟适当的疾病修正治疗而使患者的预后恶化。患有 AATD 相关肺部疾病的患者会出现与其他阻塞性肺部疾病相似的症状,并且经常被误诊多年。除了现有的筛查指南外,我们建议将 AATD 的筛查作为过敏症专家对哮喘和固定阻塞性疾病、慢性阻塞性肺疾病、无已知病因的支气管扩张症以及正在考虑使用生物制剂治疗的患者进行评估的标准部分。本文回顾了美国现有的筛查和诊断测试,并强调了基于证据的策略,以增加测试频率并提高 AATD 的检出率。我们强调了过敏症专家在管理 AATD 患者护理方面的关键作用。最后,我们敦促医疗保健提供者在 2019 冠状病毒病大流行期间注意 AATD 患者可能出现的不良临床结局。