A new study has shown that long-term oxygen therapy in patients with severe alpha-1 antitrypsin deficiency can improve survival; however, their outcomes do not improve following lung-transplantation.
Patients with severe alpha-1 antitrypsin deficiency (AATD) are at an increased risk of developing chronic obstructive pulmonary disease (COPD). Now, a new study has shown that long-term oxygen therapy (LTOT) in this patient population can improve survival; however, their outcomes do not improve following lung-transplantation.
Found in 1% to 2% of patients with COPD, the PiZZ phenotype, which is characteristic of AATD, is a significant cause for COPD. Along with vaccinations, bronchodilators, rehabilitation, and surgical interventions in selected patients, LTOT is also a treatment option.
In the current study, published in the International Journal of COPD, 14,644 patients who were on LTOT due to their COPD were enrolled. Patients with AATD, the authors note, tended to be younger, male, and never smokers. The study used data from the Swedish National Registry for Respiratory Failure for the period between January 1, 1987, and June 30, 2015.
At a median follow-up of 1.6 years on LTOT, patients without severe AATD had higher mortality (hazard ratio [HR] 1.53; 95% CI, 1.24—1.88)—there were 201 (71%) deaths among patients with AATD and 12,354 (86%) deaths among patients without AATD. The median survival was 1.9 years (95% CI, 1.8–1.9) overall; patients with AATD had a significantly longer survival (3.5 years; 95% CI, 2.8–4.3) than patients without AATD (1.9 years; 95% CI, 1.8–1.9; P<.001). This was after normalizing for age, gender, smoking status, body mass index, performance status, level of hypoxemia, and comorbidities. Cardiovascular deaths were higher in patients without AATD (16% compared with 6%; P<.001).
Of the 171 patients who underwent a lung transplant during the follow-up period, 53 (31%) had severe AATD. At a 4.4 year median follow-up after a lung transplant, 103 patients died, 36 (35%) of whom had severe AATD. While a higher proportion of AATD patients had a lung transplant—19%, compared with 1% of those without AATD—survival was similar for both populations. Higher age, according to the authors, was the only significant predictor of death after a lung transplant in this population.
Based on their findings, the authors conclude that patients with severe AATD have better survival in oxygen-dependent COPD, but prognosis following a lung transplant is independent of their AATD status.
Reference
Ekstrom M, Tanash H. Lung transplantation and survival outcomes in patients with oxygen-dependent COPD with regard to their alpha-1 antitrypsin deficiency status. Int J COPD. 2017;12: 3281-3287.
Ineligibility, Limitations to PR Uptake in Patients With AECOPD
October 15th 2024Two posters at the CHEST 2024 annual meeting revealed that 18% of eligible patients hospitalized with acute exacerbations of chronic obstructive pulmonary disease (AECOPD) participated in post-discharge pulmonary rehabilitation (PR), with ineligibility significantly limiting uptake.
Read More
A Pulmonologist on Why You Should Think About Respiratory Health and the Lungs
November 16th 2021On this episode of Managed Care Cast, we speak with MeiLan K. Han, MD, MS, the author of a book released this month called Breathing Lessons: A Doctor’s Guide to Lung Health. Han, a pulmonologist, gives an inside tour of the lungs and how they work, zooms out to examine the drivers of poor respiratory health, and addresses policy changes that are needed to improve lung health.
Listen
FDA Approves Dupilumab as First Biologic Treatment for COPD
September 27th 2024The FDA approved dupilumab today as the first biologic treatment for patients with uncontrolled chronic obstructive pulmonary disease (COPD) and an eosinophilic phenotype to significantly help reduce exacerbations and improve lung function.
Read More
Preventing Respiratory Illness and Death Through Tighter Air Quality Standards
June 1st 2021On this episode of Managed Care Cast, a research scholar at the Marron Institute of Urban Management at New York University discusses the latest findings in the Health of the Air report, which was presented at the recent American Thoracic Society 2021 International Conference.
Listen