BACKGROUND: Sex and gender influence many aspects of chronic obstructive pulmonary disease (COPD). Limited data are available on this topic in alpha-1 antitrypsin deficiency (AATD). We therefore aimed to investigate sex issues in the EARCO registry, a prospective, international, observational cohort study. METHODS: Baseline data from PiZZ individuals, enrolled in the registry with complete data on sex and smoking history were analysed by group comparisons and binary logistic regression analyses. RESULTS: 1283 patients with AATD, 49.3% women were analysed. Females reported less tobacco consumption (16.8±12.2 vs. 19.6±14.5 PY, p=0.006), occupational exposures towards gases, dusts or asbestos (p<0.005 each) and consumed less alcohol (5.5±7.6 vs. 8.4±10.3u/week, p<0.001). Females reported COPD (41% vs. 57%, p<0.001) and liver disease (11% vs. 20%, p<0.001) less often. However, they had a higher prevalence of bronchiectasis (24% vs. 13%, p<0.001). Despite better lung function (FEV1%pred. 73.6±29.9 vs. 62.7±29.5, p<0.001) females reported a similar symptom burden (CAT 13.4±9.5 vs. 12.5±8.9, p=ns) and exacerbation frequency (at least one in the previous year 30% vs. 26%, p=ns) compared to males. In multivariate analyses, female sex was an independent risk factor for exacerbations in the previous year OR 1.6 p=0.001 in addition to smoking history, COPD, asthma and bronchiectasis and was also identified as risk factors for symptom burden (CAT≥10) OR 1.4 p=0.014 besides age, BMI, COPD and smoking history. CONCLUSION: Men had higher rates of COPD and liver disease, women were more likely to have bronchiectasis. Women's higher symptom burden and exacerbation frequency suggest they may need tailored treatment approaches.

Department of Internal Medicine and Therapeutics University of Pavia Italy; Respiratory Diseases Division IRCCS Policlinico San Matteo Foundation Pavia Italy

Department of Pneumology and Critical Care Medicine Thoraxklinik University of Heidelberg Heidelberg Germany; Translational Lung Research Center Heidelberg Heidelberg Germany

Department of Pulmonology Hospital Clínico Universitario de Santiago Santiago de Compostela Spain; Interdisciplinary Research Group in Pulmonology Institute of Health Research of Santiago de Compostela Santiago de Compostela Spain

Department of Pulmonology University Hospital Zurich Zurich Switzerland; Faculty of Medicine University of Zurich Zurich Switzerland

Department of Respiratory Medicine and Allergology Skåne University Hospital Lund University Malmö Sweden

Institute for Applied Health Research University of Birmingham Birmingham B15 2TT United Kingdom; University Hospitals Birmingham NHS Foundation Trust Birmingham United Kingdom

Pneumologische Rehabilitation Deutsches Zentrum für Lungenforschung Philipps Universität Marburg Marburg Germany; Schön Klinik Berchtesgadener PMU Salzburg Austria

Pneumology Department Hospital Universitari Vall d'Hebron Vall d'Hebron Institut de Recerca Barcelona Spain

Pneumology Department Hospital Universitario Nuestra Señora de La Candelaria Santa Cruz de Tenerife Spain

Pneumology Service Hospital Alvaro Cunqueiro Vigo Institute for Health Research Galicia Sur Instituto de Salud Carlos 3 Madrid Spain

Pulmonology Department Hospital Senhora da Oliveira Guimarães Portugal

Thomayer hospital Charles University Prague Czech Republic

Unidad Médico Quirúrgica de Enfermedades Respiratorias Instituto de Biomedicina de Sevilla IBiS Hospital Universitario Virgen del Rocío CSIC Universidad de Sevilla Spain; Centro de Investigación Biomédica en Red de Enfermedades Respiratorias Instituto de Salud Carlos 3 Madrid Spain

University Medical Centre Giessen and Marburg Philipps University Deparment of Medicine Pulmonary and Critical Care Medicine Member of the German Centre for Lung Research Marburg Germany

University of Toronto Toronto Canada

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Clinical characteristics of AATD-related COPD patients vary with age at diagnosis: data from the EARCO international registry