Background: Alpha-1 antitrypsin deficiency (AATD) patients have an earlier onset of lung disease than patients with COPD not associated with AATD (usual COPD). There is a lack of recent data on AATD prevalence and disease burden of AATD with COPD (AATD-COPD) in Germany.

Aims: We describe the prevalence of AATD and the disease burden among patients with AATD-COPD and usual COPD.

Methods: In a German Sickness Fund database from 2013 to 2019, the AATD cohort was defined as ?4 claims of ICD-10 code E88.0 within a year or ?1 claim for augmentation therapy. The AATD-COPD cohort was a subset of the AATD cohort with ?2 diagnosis claims for COPD prior to AATD diagnosis. The usual COPD cohort included patients with ?4 claims for COPD within a year and were 4:1 age- and sex-matched to the AATD-COPD cohort.

Results: The point prevalence of diagnosed AATD increased from 13.3 to 30.1 per 100,000 from 2013 to 2019. At baseline, patients with AATD-COPD (n=270) were more likely to have liver disease (6% vs 2%), emphysema (46% vs 7%), and asthma (31% vs 21%) than patients with usual COPD (n=1080). At follow-up, patients with AATD-COPD had higher rates of all-cause hospitalizations (1.0 vs 0.5 per person-year), severe pulmonary exacerbations leading to hospitalization (0.2 vs 0.05 per person-year), and oxygen therapy use (24% vs 8.5%) than patients with usual COPD. For AATD-COPD vs usual COPD, the mean inpatient duration for all-cause hospitalizations was 10.2 vs 9.4 days, mean inpatient duration for hospitalizations due to severe pulmonary exacerbations was 10.7 vs 8.9 days, and 6-year mortality rate was 20% vs 13%.

Conclusion: Patients diagnosed with AATD-COPD have a higher disease burden than patients with usual COPD.