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The Antielastase Screen of the Lower Respiratory Tract of Alpha 1 Proteinase Inhibitor-sufficient Patients with Emphysema or Pneumothorax

1990; American Thoracic Society; Volume: 141; Issue: 4_pt_1 Linguagem: Inglês

10.1164/ajrccm/141.4_pt_1.880

2376-3752

Alain Gast, A Dietemann-Molard, A Pelletier, Gabrielle Pauli, Joseph G. Bieth,

Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

The present study was aimed at testing whether α1-proteinase inhibitor-sufficient patients with lung emphysema or idiopathic spontaneous pneumothorax have an impaired antielastase protection at the lung alveolar level. We have collected bronchoalveolar lavage fluids (BALF) from 20 PiMM emphysematous patients (44 ± 12 yr), 24 patients with pneumothorax but no radiologic evidence of emphysema (30 ± 11 yr), 32 healthy subjects (27 ± 6 yr), and 56 patients with sarcoidosis (30 ± 11 yr). The BALF were assayed for immunoreactive albumin, α1-proteinase inhibitor (α1PI), leukocyte elastase-α1PI complex (LE-α1PI), and mucus proteinase inhibitor (MPI) as well as for porcine pancreatic elastase inhibitory capacity, a measure of active α1PI. The healthy subjects and the patients with emphysema or pneumothorax had comparable levels of total and active α1PI and total MPI. In contrast, the levels of LE-α1PI complex were elevenfold higher in patients with emphysema than in normal subjects (p = 0.021) and tended to increase with the severity of the disease because they were negatively correlated with FEV1/FVC% (r = −0.55; 0.05 < p < 0.1). They did not vary with age in a population of patients with sarcoidosis (r = 0.03), suggesting that their elevenfold increase in emphysematous patients is not related to the age of these subjects. Patients with pneumothorax had levels of LE-α1PI complex that did not significantly differ from those of normal subjects (p = 0.24). We conclude that (1) PiMM smokers do not develop emphysema as a result of an acquired defect of active α1PI comparable to the defect of total α1PI noticed in PiZZ patients and (2) in emphysema, there is a marked increase in the rate of degranulation of neutrophils as suggested by the important increase of the levels of LE-α1PI complex.