The natural history of lung function after lung transplantation for α(1)-antitrypsin deficiency

Am J Respir Crit Care Med. 2014 Aug 1;190(3):274-81. doi: 10.1164/rccm.201401-0031OC.


Rationale: Lung transplantation (LT) is an established treatment for end-stage lung diseases, including chronic obstructive pulmonary disease (COPD) associated with α1-antitrypsin deficiency (AATD).

Objectives: We sought to compare the post-transplantation course of patients with AATD and AAT-replete COPD.

Methods: Between June 1991 and January 2008, a total of 231 patients with AAT-replete COPD and 45 with AATD underwent LT at Cleveland Clinic. Data reviewed included baseline recipient, donor, and surgical data; all spirometry evaluations; acute cellular rejection (ACR) events; and survival data. Endpoints included temporal change in FEV1, severity of ACR, and survival. A longitudinal temporal decomposition model was used for analysis.

Measurements and main results: Comparison of overall rates of FEV1 decline in AATD and AAT-replete patients with COPD showed no significant differences (P > 0.09). However, although the single LT patients had similar trends in FEV1 in both groups, patients with AATD with double LT declined faster (P < 0.002) than the AAT-replete patients. No differences in the frequency or severity of ACR episodes were observed (P = 0.32). Furthermore, there was no difference in early or late mortality between patients with AATD and patients with AAT-replete COPD (P > 0.09).

Conclusions: Although overall the post-LT FEV1 slope, severity of ACR, and survival among patients with AATD is similar to that of AAT-replete patients with COPD, patients with AATD with double LT have a faster rate of FEV1 decline. These findings support the eligibility of patients with AATD for LT, and suggest the need for additional studies to better understand the difference between single and double LT in AATD.

Keywords: FEV1 slope; lung transplantation; mixed modeling; α1-antitrypsin deficiency.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Bronchoscopy
  • Female
  • Forced Expiratory Volume
  • Humans
  • Kaplan-Meier Estimate
  • Lung Transplantation / mortality
  • Lung Transplantation / statistics & numerical data*
  • Male
  • Middle Aged
  • Ohio
  • Outcome Assessment, Health Care / statistics & numerical data
  • Pulmonary Disease, Chronic Obstructive / etiology
  • Pulmonary Disease, Chronic Obstructive / genetics
  • Pulmonary Disease, Chronic Obstructive / physiopathology
  • Pulmonary Disease, Chronic Obstructive / surgery*
  • Retrospective Studies
  • Spirometry
  • alpha 1-Antitrypsin Deficiency / complications
  • alpha 1-Antitrypsin Deficiency / genetics
  • alpha 1-Antitrypsin Deficiency / surgery*