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Traitement substitutif de l’emphysème au cours du déficit en alpha-1 antritrypsine [Augmentation therapy of alpha-1 antitrypsin deficiency associated emphysema]

Abstract : INTRODUCTION: Alpha-1 antitrypsin, secreted by the liver, inhibits neutrophil elastase. Its deficiency favours the development of emphysema. Restoring a "protective" serum level in deficient patients should make it possible to inhibit the development of emphysema. STATE OF THE ART: Human plasma-derived alpha-1 antitrypsin is a blood-derived drug sold in France under the name Alfalastin(®). The recommended posology is an I.V. administration of 60mg/kg once a week. Human plasma-derived alpha-1 antitrypsin restores anti-elastase protection in the lower lung and prevents experimental emphysema induced by the elastasis of human neutrophils in hamster. The low number of patients with alpha-1 antitrypsin deficiency is one of the difficulties to perform sufficiently powerful randomised studies. However, randomised studies have reported the efficacy of human plasma-derived alpha-1 antitrypsin perfusions on mortality, FEV1 decline and the frequency of exacerbations. Randomised control trials have demonstrated the efficacy of human plasma-derived alpha-1 antitrypsin perfusions on the loss of lung density assessed by CT scan. CONCLUSION: Augmentation therapy is simple in its conception and implementation, but it is expensive. However, there are currently no other solutions.
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https://hal-univ-rennes1.archives-ouvertes.fr/hal-01146841
Contributor : Laurent Jonchère <>
Submitted on : Wednesday, April 29, 2015 - 11:25:56 AM
Last modification on : Thursday, May 28, 2020 - 3:32:51 AM

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J. Traclet, P. Delaval, P. Terrioux, J.-F. Mornex. Traitement substitutif de l’emphysème au cours du déficit en alpha-1 antritrypsine [Augmentation therapy of alpha-1 antitrypsin deficiency associated emphysema]. Revue des Maladies Respiratoires, Elsevier Masson, 2015, 32 (4), pp.435-446. ⟨10.1016/j.rmr.2014.10.001⟩. ⟨hal-01146841⟩

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