Common variable immunodeficiency (CVID) is a major antibody-deficiency synd
rome, associated with increased risk of bacterial infection, as well as aut
oimmune and granulomatous disease. The clinical and immunological features
are heterogeneous. This heterogeneity is expressed by the case reports of t
hree selected patients. These observations will be discussed, with referenc
e to a recent classification of CVID distinguishing four different clinical
entities: i) CVID presenting with clinical and immunological features of X
-linked agammaglobulinemia; ii) CVID presenting with clinical and immunolog
ical features of X-linked hyper-IgM syndrome; iii) CVID associated with sys
temic granulomatous disease; and iiii) CVID associated with autoimmune mani
festations.