Methods: we retrospectively analyzed the records of 31 adult patients with CVID (12 female, 19 male) who were referred to our clinic by their primary care physicians between January 2010 and May 2014. European Society of Immune Deficiencies (ESID)/Pan American Group for Immune Deficiency (PAGID) diagnostic criteria were used for diagnosis of CVID. The patients were classified to clinical and immunophenotypic subgroups for the statistical comparisons. Clinical subgroups were determined according to existence of splenomegaly, hepatomegaly, lymphadenopathy, chronic complications of lower respiratory tract and chronic complications of gastrointestinal tract. Immunophenotypic subgroups were consisted of the patients had low percentage of CD19+ B cells (less than or equal to 2%) or low CD4/CD8 ratio (less than or equal to 0.63).
Results: the clinical and immunological findings of our patients were almost similar to those of the other studies available in the literature. Splenomegaly and hepatomegaly were significantly associated with each other (p=0.016) and low body weight (p=0.001, 0.013, respectively). In addition, the existace of chronic complications of gastrointestinal system (nodular lymphoid hyperplasia, villous atrophy, lymphocytic infiltrates at mucosa) was positively correlated with splenomegaly, hepatomegaly or low body weight (p=0.005, 0.045, 0.007, respectively). The patients with low body weight had lower CD4/CD8 T cell ratio than those of the patients with normal body weight (p=0.026). The patients with low percentage of CD19+ B cells had lower IgM levels but higher percentage of CD3+HLA-DR+ T cells than those of remaining patients (p = 0.035, 0.026, respectively). In addition, lower IgG levels and percentage of CD19+ B cells but higher percentage of CD3+HLA-DR+T cells were found in the patients who had low CD4/CD8 ratio than those of remaining patients (p = 0.023, 0.011, 0.028, respectively).
Conclusions: there are some relationships among clinical and immunological features of patients with CVID. However, because of relative rarity of CVID, multicenter studies should be performed to get most accurate results. Additionally, increased percentage of CD3+ T cells bearing HLA-DR in the patients with CVID suggests that the adaptive immune system is on alert and subclinical immune activation insidiously continues.