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Alphas with severe emphysema live longer after lung transplant; Swedish study finds 11-year median survival
ScienceDirect.com
A 20-year-long study in Sweden found that patients with Alpha-1 and severe emphysema survive longer with lung transplants than those who receive the usual medical therapy.
The study reported this month in The Journal of Heart and Lung Transplantation, found that Swedish Alphas survived a median of 11 years after transplant, compared to a median of eight years for those with similar disease severity who got standard medical therapy.
This is the study synopsis published online:
The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α1-anti-trypsin deficiency (PiZZ) and emphysema is still unclear. The aim of this study was to assess whether PiZZ patients who have undergone lung transplantation (the lung transplant group, TxG) do better than patients who have continued on the usual medical therapy (the non-transplant group, NTxG).
Methods:
Between 1990, when the first patient received a lung transplant in Sweden, until June 2010, a total of 83 PiZZ patients with severe emphysema underwent transplantation. Seventy appropriate controls were identified from the Swedish National AAT Deficiency Registry. Each control was matched with a patient who had received a lung transplant, for age, gender, smoking history (number of pack-years) and lung function at the time of transplantation.
Results:
Both controls and lung transplant patients had low spirometric values with a mean FEV1 of 23 ± 6% and 22 ± 9% of predicted value, respectively (not a statistically significant difference). Of the 83 transplant patients, 62 (75%) underwent single-lung transplantation (SLTx). During follow-up, 37 (45%) deaths occurred in the TxG and 45 (64%) in the NTxG. In the TxG, the estimated median survival time was 11 years (95% confidence interval [CI] 9 to 14 years), compared with 8 years (95% CI 4 to 6 years) for the NTxG (p = 0.006). The most common cause of death was pulmonary infection among the transplant patients (38%) and respiratory failure (60%) among the controls.
Conclusion:
Lung transplantation significantly improves long-term survival of patients with severe α1-anti-trypsin deficiency (PiZZ) and emphysema.
