Respiratory disorders in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infec tions, and various immunologic abnormalities. The clinical presentation is generally that of recurrent pyogenic sinopulmonary infections. Our objectives were to study the prevalence of lung involvement and the res ponse to intravenous immunoglobulin replacement therapy in 19 patients with CVID. Nineteen patients (12 men) with a mean age (SD) of 33.1 (17.1) years had a previous diagnosis of CVID and were treated with intravenous immunog lobulin replacement. All patients underwent complete pulmonary function tes ts and high-resolution computed tomography (HRCT) examination. Bronchiectas is was diagnosed in 11 (58%) patients and eight (42%) were multi-lobar bron chiectasis. Chronic airflow limitation (CAL) was present in 10 (53%) patien ts and a restrictive pattern was seen in one case. Eleven patients (58%) pr esented a decrease in single-breath carbon monoxide diffusing capacity of t he lung (DLCO). Before intravenous immunoglobulin replacement therapy (INIR T), 84% of patients had suffered from at least one episode of pneumonia. Ep isodes of lower respiratory tract infection decreased significantly from 0. 28 per patient and year before replacement therapy to 0.16 per patient and year after treatment. The mean duration of replacement therapy was 7.5 year s. In conclusion lung involvement was frequent in patients with CVID. Long-ter m administration of intravenous gammaglobulin resulted in a substantial red uction of pneumonic episodes.