TABLE 2

Prevalence of bronchiectasis adults (n=401) with any, primary, secondary or treatable immunodeficiencies according to five different sets of immunological tests (S1–S5)

S1S2S3S4S5
Any immunodeficiency36 (8.9%)93 (23.2%)179 (44.6%)179 (44.6%)179 (44.6%)
Primary immunodeficiency29 (7.2%)83 (20.7%)158 (39.4%)158 (39.4%)158 (39.4%)
 Isolated IgG subclass deficiency0036 (9%)36 (9%)36 (9%)
  •   Isolated IgG1 subclasses deficiency

003 (0.8%)3 (0.8%)3 (0.8%)
  •   Isolated IgG2 subclasses deficiency

002 (0.5%)2 (0.5%)2 (0.5%)
  •   Isolated IgG3 subclasses deficiency

006 (1.5%)6 (1.5%)6 (1.5%)
  •   Isolated IgG4 subclasses deficiency

0024 (5.9%)24 (5.9%)24 (5.9%)
  •   IgG1 and IgG3 subclasses deficiency

001 (0.2%)1 (0.2%)1 (0.2%)
 Isolated IgM deficiency003 (0.7%)3 (0.7%)3 (0.7%)
 Unclassified antibody deficiency006 (1.5%)6 (1.5%)6 (1.5%)
 CVID002 (0.5%)2 (0.5%)2 (0.5%)
 Severe combined immunodeficiency00000
 Combined immunodeficiency00000
 Hyper-IgE syndrome00000
 Isolated IgA deficiency2 (0.5%)2 (0.5%)2 (0.5%)2 (0.5%)2 (0.5%)
 DiGeorge Syndrome1 (0.2%)1 (0.2%)1 (0.2%)1 (0.2%)1 (0.2%)
 Unclassified immunodeficiency26 (6.5%)80 (20%)108 (26.9%)108 (26.9%)108 (26.9%)
Secondary immunodeficiency7 (1.7%)10 (2.5%)21 (5.2%)21 (5.2%)21 (5.2%)
 Immunosuppressive drugs1 (0.2%)3 (0.7%)11 (2.7%)11 (2.7%)11 (2.7%)
  •   Steroids

1 (0.2%)3 (0.7%)8 (2%)8 (2%)8 (2%)
  •   Biologics

0 (0%)0 (0%)2 (0.5%)2 (0.5%)2 (0.5%)
  •   Antiproliferative agents

0 (0%)0 (0%)1 (0.2%)1 (0.2%)1 (0.2%)
 Haematological malignancy6 (1.5%)6 (1.5%)7 (1.7%)7 (1.7%)7 (1.7%)
 Transplant01 (0.2%)3 (0.7%)3 (0.7%)3 (0.7%)
Other aetiologies113 (28.2%)113 (28.2%)67 (16.7%)67 (16.7%)67 (16.7%)#
Idiopathic bronchiectasis252 (62.8%)195 (48.6%)155 (38.7%)155 (38.7%)155 (38.7%)
Treatable immunodeficiencies15 (3.7%)37 (9.2%)67 (16.7%)67 (16.7%)67 (16.7%)

S1: complete blood count, and total IgG, IgA and IgM serum levels; S2: S1 plus IgG subclasses; S3: S2 plus lymphocyte subsets; S4: S3 plus total IgE; S5: S4 plus HIV testing. CVID: common variable immunodeficiency. #: including 26 post-infective, eight primary ciliary dyskinesia and five allergic bronchopulmonary aspergillosis.