Table 1.

CVID criteria according to the ESID guidelines. So far, CVID has been diagnosed as probable or possible according to the ESID criteria [20]. In 2016, these criteria were updated. At the moment CVID can be diagnosed in the following situations

I	The occurrence of at least one of the following: increased susceptibility to infection, autoimmune manifestations, granulomatous disease, unexplained polyclonal lymphoproliferation, positive family history
II	And decreased IgG and IgA levels, with or without the decrease of IgM level (measured at least twice, less than 2 standard deviations of the normal levels for the age)
III	And at least one of the following: abnormal, poor response to vaccines and/or absence of isohemagglutinins, absence of the protective level of specific antibodies despite vaccination, low switched memory B-cells (less than 70% of the age-related normal value)
IV	And exclusion of secondary causes of hypogammaglobulinaemia
V	And a diagnosis after the age of four, although clinical symptoms may occur earlier
VI	And no evidence of profound T-cell deficiency