Diagnosis and Monitoring Pathways Using Non‐Invasive Tests in Patients With Alpha‐1 Antitrypsin Deficiency‐Associated Liver Disease: Results From an Expert Delphi Panel

作者：Virginia Clark, Mark Price, Jon Russo, Rohit Loomba, Alice Turner, Pavel Strnad · 发表于：United European Gastroenterology Journal · 年份：2025 · DOI：10.1002/ueg2.70009 · 被引用次数：5 · 研究领域：Liver Disease and Transplantation、Protease and Inhibitor Mechanisms、Thyroid Disorders and Treatments

BACKGROUND AND AIMS: The severe alpha-1 antitrypsin deficiency (AATD) genotype Pi*ZZ increases the risk of liver disease (AATD-LD) and lung disease. While non-invasive tests (NITs) are widely used for fibrosis stage and monitoring of all liver diseases, the consensus for use in AATD-LD is limited. A Delphi panel study was conducted to address this need. METHOD: Healthcare providers who managed at least two patients with AATD-LD in the past two years participated. Two iterative surveys were developed and administered. The second survey clarified the results from the first and provided deeper feedback. As follow-up, a real-time consensus-building exercise focused on survey topics without consensus. Controlled feedback was anonymous. RESULTS: A total of 20 AATD experts (hepatology [n = 9], pulmonology [n = 6], transplant hepatology [n = 3], gastroenterology [n = 1], and hepatology and transplant hepatology [n = 1]) completed the study. A strong consensus was achieved around the use and evaluation of NITs for risk stratification and monitoring. All panelists agreed that vibration-controlled transient elastography (VCTE) is the preferred NIT for the initial assessment of AATD-LD owing to its accessibility and reliability. Magnetic resonance elastography and enhanced liver fibrosis tests were also considered valuable. Most (85%) agreed that VCTE < 8 kPa could indicate no or mild fibrosis and VCTE ≥ 8 kPa could indicate clinically significant fibrosis, which may correspond to fibros...