Ekstrom M, et al. - This prospective, population-based, consecutive cohort study was designed to assess the outcomes during long-term oxygen therapy (LTOT) in patients with severe alpha-1 antitrypsin deficiency (AATD) and hypoxemia. Findings demonstrated that in oxygen-dependent COPD, patients with severe AATD had a longer survival time on LTOT, however, lung transplantation in these patients afforded a similar prognosis as compared with patients without AATD.

Methods

• Researchers performed a prospective, population-based, consecutive cohort study.
• Participants included patients on LTOT due to COPD in the period from January 1, 1987, to June 30, 2015, in the Swedish National Registry for Respiratory Failure (Swedevox).
• They used the Swedish AATD registry to identify severe AATD and isoelectric focusing for confirmation.
• They gathered data on lung transplantation (LTx) from the two lung transplantation centers in Sweden.
• They assessed mortality and causes of death based on the National Causes of Death Registry and used multivariable Cox regression for analysis.

Results

• This analysis included a total of 14,644 patients who started LTOT due to COPD.
• Data reported that no patient was lost to follow up.
• Researchers noted that patients with AATD were younger, included more males and more never smokers, and had fewer comorbidities.
• Findings also demonstrated that during a median follow-up of 1.6 years (interquartile range [IQR], 2.7) on LTOT, patients without severe AATD had a higher mortality, hazard ratio [HR] 1.53 (95% CI, 1.24–1.88), adjusting for age, sex, smoking status, body mass index, performance status, level of hypoxemia, and comorbidities.
• An increment in cardiovascular deaths was documented.
• According to results, a higher proportion of AATD patients underwent LTx, 53 (19%) vs 118 (1%).
• Additionally, researchers found that post-LTx survival was similar for AATD and non-AATD patients and was predicted by age.