Systematic literature review of pulmonary function decline in AATD-associated liver/lung disease

<bold>Background/Aim:</bold> Pulmonary function (PF) decline in patients with alpha-1 antitrypsin deficiency (AATD)-associated lung disease (AATD-LgD) and/or liver disease (AATD-LD) is poorly understood. A systematic literature review investigated PF decline in patients with AATD-LgD and/or LD. <bold>Methods:</bold> Following PRISMA guidelines, relevant studies were identified using MEDLINE/Embase (2003–2023); key congresses were hand-searched (2021–2023). Randomized controlled trials (RCTs) and observational studies that reported PF tests in patients with AATD-LgD and/or LD were included. Full-text articles were assessed for quality using relevant Joanna Briggs Institute tools. Studies that evaluated change from baseline forced expiratory volume in 1 second (FEV1; percent predicted value [%] or in mL) in patients without intervention (including augmentation therapy or liver transplantation) are reported herein. <bold>Results:</bold> Four RCTs and three observational studies reported an overall decline from baseline FEV1 in patients with AATD-LgD (<bold>Table 1</bold>). No RCTs/observational studies evaluated change from baseline FEV1 for patients with AATD-LgD and LD, or AATD-LD. <bold>Conclusions:</bold> In patients with AATD-LgD, there was variability across studies in PF decline (as measured by FEV1) from baseline to Month 6 through Year 3. Additional research in patients with AATD-LgD and LD, and AATD-LD, is required to fully characterize PF decline in these cohorts. <fig><object-id>erj;64/suppl_68/PA368/F1</object-id><object-id>F1</object-id><object-id>F1</object-id><graphic></graphic></fig>