Is Standard COPD Therapy Effective in Alpha-1 Antitrypsin Deficiency?

Intravenous augmentation may slow the progression of emphysema in patients with alpha-1 antitrypsin deficiency (AATD), but other treatment options for non-AATD-related chronic obstructive pulmonary disease (COPD) may not be effective in these patients, according to a recent study.

AATD is a genetic condition that predisposes patients to COPD, and its treatment in generally extrapolated from COPD unrelated to AATD, according to the researchers.
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For their meta-analysis, the researchers included 52 trials with 5632 participants, of which 26 studies involved alpha-1 antitrypsin augmentation and 17 involved surgical treatment options.

The researchers grouped the studies into 1 of 4 categories: COPD medical, COPD surgical, AATD specific, and other. Most of the studies used CT density, forced expiratory volume in 1 s, diffusing capacity of the lungs for carbon monoxide, health status, and exacerbation rates as their outcomes.

Overall, meta-analyses were only possible for randomized controlled trials involving intravenous augmentation, which slowed progression of emphysema as measured by CT density change and was associated with a small increase in exacerbations (0.29/year). Mortality following lung transplant was similar between AATD- and non-AATD-related COPD.

“Intravenous augmentation remains the only disease-specific therapy in AATD and there is evidence that this slows decline in emphysema determined by CT density. There is paucity of data around other treatments in AATD. Treatments for usual COPD may not be as efficacious in AATD, and further studies may be required for this disease group,” the researchers concluded.

—Michael Potts

Reference:

Edgar RG, Patel M, Bayliss S, et al. Treatment of lung disease in alpha-1 antitrypsin deficiency: a systematic review [published online May 2, 2017]. Int J COPD. doi:https://doi.org/10.2147/COPD.S130440.