Am. J. Respir. Crit. Care Med., Vol 149, No. 4, Apr 1994, 966-971.
The proteinase-antiproteinase balance in alpha-1-proteinase inhibitor- deficient lung transplant recipients
MB King, EJ Campbell, BH Gray and MI Hertz
Department of Internal Medicine, University of Minnesota Medical School, Minneapolis 55455.
We examined the proteinase-antiproteinase balance in the bronchoalveolar
lavage (BAL) fluid from alpha-1-proteinase inhibitor (alpha 1PI)-deficient
lung transplant recipients to determine whether they would derive benefit
from intravenous augmentation therapy with alpha 1PI. BAL fluid from 11
alpha 1PI-deficient lung transplant recipients and eight control subjects
was assayed for free neutrophil elastase activity, immunoreactive alpha
1PI, and elastase inhibitory capacity. Samples were obtained during
intervals of health and respiratory illness. BAL fluid from healthy alpha
1PI-deficient lung transplant recipients had minimal or unmeasurable free
elastase activity, which was not different from that of control subjects.
alpha 1PI concentrations in BAL fluid from alpha 1PI-deficient lung
transplant recipients were reduced when compared with those of control
subjects. Despite this observation, all but one alpha 1PI-deficient patient
had the ability to inhibit exogenous elastase. During respiratory illness,
however, three of seven alpha 1PI-deficient lung transplant recipients had
measurable free elastase activity, which was inhibited ex vivo by addition
of alpha 1PI. We conclude that alpha 1PI- deficient lung transplant
recipients demonstrate free elastase activity in BAL fluid during severe
lower respiratory tract inflammation, which is not present during health.
Intravenous supplementation of alpha 1PI- deficient lung transplant
recipients with exogenous alpha 1PI during respiratory tract inflammation
may be indicated to inhibit elastase- mediated injury to the transplanted
lung.
