Common variable immunodeficiency is the most common symptomatic primary imm
unodeficiency disease. The patients typically present with a long history o
f respiratory tract infections, sometimes sarcoid-like lesions and in rare
cases boils. Heterogeneity of initial clinical manifestations as well as in
sufficient knowledge of the syndrome often delay the diagnosis. However, ea
rly therapy is important to reduce infections and in particular the develop
ment of bronchiectasis. Documenting 19 cases, we discuss initial clinical m
anifestations, some clinical complications, diagnostic procedures and thera
peutic management.