Diagnostic findings in 95 Finnish patients with common variable immunodeficiency

Common variable immunodeficiency is the most frequent of the primary hypoga mmaglobulinemias. It is manifested by a wide variety of clinical signs and symptoms. In this retrospective, nationwide survey data were collected on a ll patients with common variable immunodeficiency who were receiving immuno globulin replacement therapy in Finland to study the prediagnostic clinical picture, diagnostic delay, and diagnostic findings. Ninety-five patients w ere identified. The median age of the patients was 33 years. Sixteen of the patients were children. Sinopulmonary infections were the most common pred iagnostic signs and symptoms; 66% had suffered from recurrent pneumonia, 60 % from recurrent maxillary sinusitis, and 45% from recurrent bronchitis. Th ere was a considerable delay in diagnosis. The mean delay was 8 years. At t he time of diagnosis chronic pulmonary complications had already developed in 17% of the patients. The diagnosis was based on low serum immunoglobulin concentrations. This study showed that the awareness of common variable im munodeficiency is low. To improve the recognition of hypogammaglobulinemia, it should be suspected in every patient with recurrent bacterial infection s. In addition to a low serum IgG concentration, measurement of specific an tibody production is recommended to establish the diagnosis before initiati on of a life-long and costly replacement therapy.