Advanced

Survival benefit of lung transplantation in individuals with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema.

Tanash, Hanan LU ; Riise, Gerdt C; Hansson, Lennart LU ; Nilsson, Peter LU and Piitulainen, Eeva LU (2011) In The Journal of Heart and Lung Transplantation 30(12). p.1342-1347
Abstract
BACKGROUND:

The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α(1)-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... (More)
BACKGROUND:

The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α(1)-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 FEV(1) 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 α(1)-anti-trypsin deficiency (PiZZ) and emphysema. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
alpha(1)-anti-trypsin deficiency, lung transplantation, survival, mortality, COPD, emphysema, causes of death
in
The Journal of Heart and Lung Transplantation
volume
30
issue
12
pages
1342 - 1347
publisher
Elsevier
external identifiers
  • wos:000297385400006
  • pmid:21821433
  • scopus:80655147089
ISSN
1557-3117
DOI
10.1016/j.healun.2011.07.003
language
English
LU publication?
yes
id
a3426c3f-651e-480b-9660-c1ed1e5341f2 (old id 2151358)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/21821433?dopt=Abstract
date added to LUP
2011-09-04 21:21:41
date last changed
2017-10-08 03:08:10
@article{a3426c3f-651e-480b-9660-c1ed1e5341f2,
  abstract     = {BACKGROUND:<br/><br>
The objective of lung transplantation (LTx) is to prolong life, but the survival benefit for patients with severe α(1)-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).<br/><br>
<br/><br>
METHODS:<br/><br>
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.<br/><br>
<br/><br>
RESULTS:<br/><br>
Both controls and lung transplant patients had low spirometric values with a mean FEV(1) 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.<br/><br>
<br/><br>
CONCLUSION:<br/><br>
Lung transplantation significantly improves long-term survival of patients with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema.},
  author       = {Tanash, Hanan and Riise, Gerdt C and Hansson, Lennart and Nilsson, Peter and Piitulainen, Eeva},
  issn         = {1557-3117},
  keyword      = {alpha(1)-anti-trypsin deficiency,lung transplantation,survival,mortality,COPD,emphysema,causes of death},
  language     = {eng},
  number       = {12},
  pages        = {1342--1347},
  publisher    = {Elsevier},
  series       = {The Journal of Heart and Lung Transplantation},
  title        = {Survival benefit of lung transplantation in individuals with severe α(1)-anti-trypsin deficiency (PiZZ) and emphysema.},
  url          = {http://dx.doi.org/10.1016/j.healun.2011.07.003},
  volume       = {30},
  year         = {2011},
}