Ameratunga et al reported criteria for the diagnosis of Common Variable Immune Deficiency (CVID). The authors are from Auckland Hospital, Royal Melbourne Hospital and Royal Brisbane Hospital.
Major criteria - all of the following:
(1) hypogammaglobulinemia for IgG (IgG < 5 g/L)
(2) no other cause for immune defects
(3) age at diagnosis > 4 years
Sequelae due to immune system failure - one or more of the following:
(1) recurrent, severe or unusual infections
(2) poor response to appropriate antibiotic therapy
(3) breakthrough infections despite prophylactic antibiotics
(4) infection despite appropriate vaccination
(5) bronchiectasis and/or chronic sinus disease
(6) inflammatory disorder or autoimmunity
Supportive laboratory finding - 3 or more of the following:
(1) reduction of IgA and/or IgM (< 0.8 g/L for IgA; < 0.4 g/L for IgM)
(2) reduced memory B cell subsets and/or increased low CD21 subsets by flow cytometry
(3) IgG3 deficiency (< 0.3 g/L)
(4) impaired vaccine response compared with age-matched controls
(5) absent isohemagglutinins if blood type A, B or O
(6) serologic evidence of significant autoimmunity (Coombs positive, other)
(7) gene associated with CVID (TACI, BAFFR, MSH5, etc)
Histologic marker: - one or more of the following:
(1) lymphoid interstitial pneumonia
(2) granulomatous disorder
(3) nodular regenerative hyperplasia of the liver
(4) nodular lymphoid hyperplasia of the intestine
(5) absence of plasma cells in GI biopsy
CVID is probable if the patient meets:
(1) all of the major criteria
(2) 1 or more of the sequelae
(3) supportive laboratory and/or histologic markers
