title: [Small airway diseases and immune deficiency].
abstract: BACKGROUND
  Innate or acquired immune deficiency may show respiratory manifestations, often characterized by small airway involvement. The purpose of this article is to provide an overview of small airway disease across the major causes of immune deficiency.
  
  
  BACKGROUND
  In patients with common variable immune deficiency, recurrent lower airway infections may lead to bronchiolitis and bronchiectasis. Follicular and/or granulomatous bronchiolitis of unknown origin may also occur. Bronchiolitis obliterans is the leading cause of death after the first year in patients with lung transplantation. Bronchiolitis obliterans also occurs in patients with allogeneic haematopoietic stem cell transplantation, especially in the context of systemic graft-versus-host disease.
  
  
  CONCLUSIONS
  Small airway diseases have different clinical expression and pathophysiology across various causes of immune deficiency. A better understanding of small airways disease pathogenesis in these settings may lead to the development of novel targeted therapies.
authors: Université Paris Descartes, Sorbonne Paris Cité, 75005 Paris, France; Service de pneumologie, hôpital Cochin, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France. Electronic address: pierre-regis.burgel@cch.aphp.fr.
authors: P-R
authors: PR
authors: Burgel
authors: Université Paris Diderot, Sorbonne Paris Cité, 75013 Paris, France; Service de pneumologie, hôpital Saint-Louis, AP-HP, 75010 Paris, France.
authors: A
authors: A
authors: Bergeron
authors: Department of Chest Medicine, Erasme University Hospital, université libre de Bruxelles, Bruxelles, Belgique.
authors: C
authors: C
authors: Knoop
authors: Université Paris Descartes, Sorbonne Paris Cité, 75005 Paris, France; Service de pneumologie, hôpital Cochin, AP-HP, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
authors: D
authors: D
authors: Dusser
mesh_terms: D001982:Bronchial Diseases / Q000453:epidemiology / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D001989:Bronchiolitis Obliterans / Q000453:epidemiology / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000453:epidemiology / Q000473:pathology; D016040:Lung Transplantation / Q000009:adverse effects
keywords: Bronchiolite oblitérante; Bronchiolitis obliterans; Common variable immune deficiency; Déficit immunitaire commun variable; Granulomatose; Granulomatous disease; Greffe; Transplantation