Table. 1—

Aetiology of bronchiectasis

AetiologySubjects nFEV1FVC
Immunodeficiency4675 (15–133)76 (14–122)
 Primary immune defects
  Hyper-IgE syndrome2
  Mannose-binding protein deficiency1
  Qualitative antibody deficiency1
  IgG deficiency4
  Combined variable immunodeficiency14
  Undefined combined immunodeficiency6
  X-linked agammaglobulinaemia4
  MHC class-2 deficiency1
  B-cell deficiency1
  Chronic mucocutaneous candidiasis1
  Chronic granulomatous disease1
  Wiskott–Aldrich syndrome1
 Secondary immune defects
Idiopathic3569 (34–104)78 (51–116)
Aspiration2571 (33–91)69 (36–105)
Primary ciliary dyskinesia2066 (39–100)82 (46–111)
Childhood respiratory infection539 (26–62)64 (42–76)
Congenital structural malformation583 (78–85)92 (84–98)
 Congenital lobar emphysema1
 Pulmonary artery sling1
 Bronchial atresia with bronchocoele1
 Yellow nail syndrome1
  • Data are presented as median percentage predicted (range), unless otherwise stated. FEV1: forced expiratory volume in one second; FVC: forced vital capacity; Ig: immunoglobulin; MHC: major histocompatibility complex.