Research Project

Early-Life Determinants of Lung Disease in Severe Alpha-1 Antitrypsin Deficiency: the Role of Prematurity in the EARCO Registry

Principal Investigator:

Davide Piloni

Center:

University of Pavia / Fondazione IRCCS Policlinico San Matteo

City/Country:

Pavia, Italy

Start date:

September 2026

Status:

Ongoing

Contact E-mail:

d.piloni@smatteo.pv.it

Introduction

Prematurity is increasingly recognized as a determinant of altered lung growth and reduced maximally attained lung function in adulthood, even in individuals without known genetic respiratory disorders. Subjects born preterm may exhibit persistent structural and functional abnormalities, including impaired alveolarization, reduced lung volumes, airway obstruction, and increased susceptibility to respiratory disease later in life. However, the potential interaction between prematurity and severe AATD has never been systematically investigated.

Objectives

The primary objective of the study is to evaluate whether prematurity is associated with different lung function trajectories and increased respiratory disease burden in severe AATD patients enrolled in the EARCO registry.

Inclusion criteria

Adult participants in the EARCO Registry

Brief summary

Participants will be primarily classified as born preterm (<37 completed weeks of gestation) or at term (≥37 weeks). Where the available sample size allows, prematurity will be further categorised as extremely preterm (<28 weeks), very preterm (28 to <32 weeks), and moderate-to-late preterm (32 to <37 weeks). Baseline demographic, clinical and functional characteristics will be described and compared according to prematurity status. Longitudinal analyses will assess whether prematurity is associated with different trajectories of lung function, particularly FEV₁ and DLCO, as well as with respiratory outcomes over time, including exacerbations and disease burden