[Late manifestation of common variable immunodeficiency by Biermer's disease]

Common variable immunodeficiency (CVI) is a heterogeneous disorder characterized by decreased production of antibodies. Clinical presentation of CVI is generally that of recurrent pyogenic infections. Autoimmune diseases can also occur. The age of onset of symptoms shows two peaks at 1-5 and 16-20 years.

A 77-year-old man was admitted in hospital for pernicious anemia. We discovered hypogammaglobulinemia with low levels of immunoglobulin G, A and M, but normal T-cell levels. We diagnosed common variable immunodeficiency. This patient had not had recurrent pyogenic infections.

This case shows that common variable immunodeficiency can be revealed late by an autoimmune disease. The pathogenesis of autoimmune diseases in this immunodeficiency remains unknown despite several possible explanations.