Predicting Exacerbations in Alpha-1 Antitrypsin Deficiency Using Clinical and Pulmonary Function Tests: Portuguese EARCO Registry.

IF 3.5 3区医学 Q2 RESPIRATORY SYSTEM

Respiration Pub Date : 2024-01-01 Epub Date: 2024-03-26 DOI:10.1159/000537759

Nuno Faria, Joana Gomes, Catarina Guimarães, Raquel Marçôa, Beatriz Ferraz, Maria Sucena

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引用次数: 0

Abstract

Introduction: Exacerbations are common in individuals with alpha-1 antitrypsin deficiency (AATD)-related lung disease. This study intended to identify independent predictive factors for exacerbations in AATD using the Portuguese European Alpha-1 Research Collaboration (EARCO) registry.

Methods: This study includes patients from the Portuguese EARCO registry, a prospective multicenter cohort (NCT04180319). From October 2020 to April 2023, this registry enrolled 137 patients, 14 of whom were excluded for analysis for either missing 12 months of follow-up or baseline pulmonary function.

Results: Among the 123 AATD patients, 27 (22.0%) had at least one exacerbation in the last 12 months of follow-up. Patients with Pi*ZZ phenotype were three times more likely than the rest of the population to experience any exacerbation (32.7 vs. 14.1%, p = 0.014; OR 3.0). BODE index was significantly higher in exacerbators than in non-exacerbators (3.9 ± 2.4 vs. 1.3 ± 1.2; p < 0.001), including on multivariate analysis (p = 0.002). Similar results were found for BODEx (multivariate p < 0.001). DLCO was the only functional parameter independently associated with exacerbations (p = 0.024).

Conclusions: DLCO, BODE, and BODEx were independent predictors of exacerbations at 12 months in AATD patients. Understanding these risk factors can aid decision-making on AATD-related lung disease management and improve patient outcomes.

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利用临床和肺功能测试预测α1-抗胰蛋白酶缺乏症的病情恶化：葡萄牙 EARCO 登记。

导言：在α-1-抗胰蛋白酶缺乏症（AATD）相关肺部疾病患者中，病情加重很常见。本研究旨在利用葡萄牙欧洲α-1研究合作组织（EARCO）登记资料，确定α-1-抗胰蛋白酶缺乏症肺部疾病恶化的独立预测因素：本研究包括葡萄牙 EARCO 登记处的患者，这是一项前瞻性多中心队列研究 (NCT04180319)。从 2020 年 10 月到 2023 年 4 月，该登记处共登记了 137 名患者，其中 14 名患者因缺少 12 个月的随访或基线肺功能而被排除在分析之外：在 123 名 AATD 患者中，有 27 人（22.0%）在过去 12 个月的随访中至少出现过一次病情加重。Pi*ZZ表型患者出现病情加重的几率是其他患者的三倍（32.7% vs 14.1%，P=0.014；OR 3.0）。病情加重者的 BODE 指数明显高于非病情加重者（3.9±2.4 vs 1.3±1.2；p 结论：DLCO、BODE和BODEx是AATD患者12个月后病情加重的独立预测因素。了解这些风险因素有助于做出与 AATD 相关的肺部疾病管理决策，改善患者的预后。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Respiration 医学-呼吸系统

CiteScore

7.30

自引率

5.40%

发文量

审稿时长

4-8 weeks

期刊介绍： ''Respiration'' brings together the results of both clinical and experimental investigations on all aspects of the respiratory system in health and disease. Clinical improvements in the diagnosis and treatment of chest and lung diseases are covered, as are the latest findings in physiology, biochemistry, pathology, immunology and pharmacology. The journal includes classic features such as editorials that accompany original articles in clinical and basic science research, reviews and letters to the editor. Further sections are: Technical Notes, The Eye Catcher, What’s Your Diagnosis?, The Opinion Corner, New Drugs in Respiratory Medicine, New Insights from Clinical Practice and Guidelines. ''Respiration'' is the official journal of the Swiss Society for Pneumology (SGP) and also home to the European Association for Bronchology and Interventional Pulmonology (EABIP), which occupies a dedicated section on Interventional Pulmonology in the journal. This modern mix of different features and a stringent peer-review process by a dedicated editorial board make ''Respiration'' a complete guide to progress in thoracic medicine.