In 2014 the European Society for Immunodeficiencies (ESID) reported revised criteria for the diagnosis of Common Variable Immunodeficiency Disorder (CVID).
Criteria for CVID - all of the following:
(1) at least one of the following
(1a) increased susceptibility to infection
(1b) autoimmune manifestations
(1c) granulomatous disease
(1d) unexplained polyclonal lymphoproliferation
(1e) affected family member with antibody deficiency
(2) marked decrease of IgG and IgA with or without low IgM levels
(3) at least one of the following:
(3a) poor antibody response to vaccines (absence of protective levels despite vaccination)
(3b) absent isohemagglutinins
(3c) low switched memory B cells (less than 70% of age-related normal values)
(4) exclusion of secondary causes of hypogammaglobulinemia
(5) diagnosis established after the age of 4 (symptoms may start sooner)
(6) no evidence of profound T-cell deficiency
where:
• Marked decrease in immunoglobulins: more than 2 SD below mean for age.
• Measurement of immunoglobulins should be done 2 or more times.
• Features of T-cell deficiency: (1) absent T-cell proliferation; (2) low percent naive CD4-positive cells (< 25% if 2 to 6 years old; < 20% 6 to 16 years old; < 10% over 16 years old); (3) low absolute CD4 positive cells (< 300 per µL 2 to 6 years old; < 250 per µL 6 to 12 years old; < 200 per µL if over 12 years old).
