Sex-Differences in Alpha-1 Antitrypsin Deficiency: Data From the EARCO Registry

Abstract

Alpha1-antitrypsin deficiency; Chronic obstructive pulmonary disease; Gender

Deficiència d'alfa1-antitripsina; Malaltia pulmonar obstructiva crònica; Gènere

Deficiencia de alfa1-antitripsina; Enfermedad pulmonar obstructiva crónica; Género

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 ± 10.3 u/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.

The International EARCO Registry is funded by unrestricted grants from Grifols, CSL Behring, Kamada, pH Pharma and Takeda to the European Respiratory Society (ERS). AMT is funded by the NIHR Midlands Patient Safety Research Collaboration (PSRC) and Applied Research Collaborative West Midlands. She has also received grant funding from NIHR HTA and EME. The views expressed are those of the author(s) and not necessarily those of the NIHR or the Department of Health and Social Care.