par Burgel, Pierre-Régis;Bergeron, Anne;Knoop, Christiane 
;Dusser, Daniel
Référence Revue des maladies respiratoires, 33, 2, page (145-155)
Publication Publié, 2016-02
;Dusser, DanielRéférence Revue des maladies respiratoires, 33, 2, page (145-155)
Publication Publié, 2016-02
Article révisé par les pairs
| Résumé : | Introduction. - Innate or acquired immune deficiency may show respiratory manifestations,often characterized by small airway involvement. The purpose of this article is to provide anoverview of small airway disease across the major causes of immune deficiency.Background. - In patients with common variable immune deficiency, recurrent lower airwayinfections may lead to Bronchiolitis and Bronchiectasis. Follicular and/or granulomatous Bron-chiolitis of unknown origin may also occur. Bronchiolitis obliterans is the leading cause of deathafter the first year in patients with lung transplantation. Bronchiolitis obliterans also occurs inpatients with allogeneic haematopoietic stem cell transplantation, especially in the context ofsystemic graft-versus-host disease.Viewpoint and conclusion. - Small airway diseases have different clinical expression and patho-physiology across various causes of immune deficiency. A better understanding of small airwaysdisease pathogenesis in these settings may lead to the development of novel targeted therapies. |
