title: [Lung disease in adult common variable immunodeficiency].
abstract: BACKGROUND
  Common variable immunodeficiency (CVID) is characterized by a defect in antibody production and may be complicated by infectious or non-infectious respiratory disease.
  
  
  BACKGROUND
  In addition to recurrent infectious complications, mainly due to encapsulated bacteria, CVID may be complicated by diffuse infiltrative, non-infectious lung disease. The latter may be related to granulomatosis, lymphoid interstitial pneumonia, follicular bronchiolitis, follicular nodular hyperplasia, organizing pneumonia or lymphoma. Different lymphoid histological lesions can co-exist and form a new entity called GLILD (granulomatous lymphocytic interstitial lung disease), which is associated with a poor prognosis. Replacement of immunoglobulins significantly decreases the frequency and severity of infections but has no impact on the non-infectious complications.
  
  
  CONCLUSIONS
  Studies are needed to determine the modalities of follow-up and better understand the long-term progress of GLILD. These studies should improve the management of GLILD in the context of immunosuppressive treatments, which increase the risk of infection in CVID.
  
  
  CONCLUSIONS
  The identification of GLILD, which reflects a variable histological spectrum, rather than a well-defined entity, necessitates revising the approach to diffuse infiltrative lung diseases in CVID.
authors.affiliation: U1153 CREBS, Biostatistics and Clinical Epidemiology Research Team, service de pneumologie, hôpital Saint-Louis, AP-HP, université Paris Diderot, Sorbonne Paris Cité, 75010 Paris, France.
authors.forename: J
authors.initials: J
authors.lastname: Hadjadj
authors.affiliation: Département d'immuno-hématologie, hôpital Saint-Louis, AP-HP, 75010 Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Malphettes
authors.affiliation: Département d'immuno-hématologie, hôpital Saint-Louis, AP-HP, 75010 Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Fieschi
authors.affiliation: Département d'immuno-hématologie, hôpital Saint-Louis, AP-HP, 75010 Paris, France.
authors.forename: E
authors.initials: E
authors.lastname: Oksenhendler
authors.affiliation: U1153 CREBS, Biostatistics and Clinical Epidemiology Research Team, service de pneumologie, hôpital Saint-Louis, AP-HP, université Paris Diderot, Sorbonne Paris Cité, 75010 Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Tazi
authors.affiliation: U1153 CREBS, Biostatistics and Clinical Epidemiology Research Team, service de pneumologie, hôpital Saint-Louis, AP-HP, université Paris Diderot, Sorbonne Paris Cité, 75010 Paris, France. Electronic address: anne.bergeron-lafaurie@sls.aphp.fr.
authors.forename: A
authors.initials: A
authors.lastname: Bergeron
mesh_terms: D000328:Adult; D017074:Common Variable Immunodeficiency / Q000150:complications*; D006801:Humans; D008171:Lung Diseases / Q000175:diagnosis / Q000209:etiology*; D017563:Lung Diseases, Interstitial / Q000209:etiology
keywords: Bronchiectasis; Common variable immunodeficiency; Dilatation des bronches; Déficit immunitaire commun variable; GLILD; Granulomatose; Granulomatous disease; Lymphoma; Lymphome