Development of pulmonary abnormalities in patients with common variable immunodeficiency: associations with clinical and immunologic factors

Abstract

Patients with common variable immunodeficiency (CVID) have low serum IgG, IgA, and/or IgM levels and recurrent airway infections. Radiologic pulmonary abnormalities and impaired function are common complications. It is unclear to what extent IgG replacement treatment prevents further pulmonary damage and how factors beside infections may contribute to progression of disease. To study the development of pulmonary damage and determine how clinical and immunologic factors, such as serum IgG, may contribute to possible changes. In a retrospective, longitudinal study of 54 patients with CVID already treated with immunoglobulins, we examined changes of lung function and findings on high-resolution computed tomography (HRCT), obtained at 2 time points (the date of the last pulmonary function measurement before April 2005 [T1] and the date of the measurement performed closest to 5 years earlier [T0]) 2 to 7 years apart and explored possible relations to clinical and immunologic factors such as levels of IgG, tumor necrosis alpha (TNF-alpha), and mannose-binding lectin (MBL) in serum. Despite a mean (SD) serum IgG level of 7.6 (2.3) g/L for all the patients during the entire study period, lung function decreased from T0 to T1. The combination of a low serum IgA level and serum MBL was associated with the presence of bronchiectasis and lower lung function and with worsening of several HRCT abnormalities from T0 to T1. Increased serum levels of TNF-alpha were related to deterioration of gas diffusion. A mean serum IgG level less than 5 g/L between T0 and T1 was associated with worsening of linear and/or irregular opacities seen on HRCT. For a period of 4 years, lung function and HRCT deteriorated in CVID patients treated with immunoglobulins.