Respiratory disorders in common variable immunodeficiency

Abstract

Common variable immunodeficiency (CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, 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 response 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 immunoglobulin replacement. All patients underwent complete pulmonary function tests and high-resolution computed tomography (HRCT) examination. Bronchiectasis was diagnosed in 11 (58%) patients and eight (42%) were multi-lobar bronchiectasis. Chronic airflow limitation (CAL) was present in 10 (53%) patients and a restrictive pattern was seen in one case. Eleven patients (58%) presented a decrease in single-breath carbon monoxide diffusing capacity of the lung (DL(CO)). Before intravenous immunoglobulin replacement therapy (INIRT), 84% of patients had suffered from at least one episode of pneumonia. Episodes 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 years. In conclusion lung involvement was frequent in patients with CVID. Long-term administration of intravenous gammaglobulin resulted in a substantial reduction of pneumonic episodes.

Tertiary lymphoid neogenesis is a component of pulmonary lymphoid hyperplasia in patients with common variable immunodeficiency

“…16,28–30 PLH represents a clinically relevant proportion of CVID-associated lung disease because nearly 40% of patients with CVID with respiratory symptoms and radiographic findings had PLH in a large study. 7 CVID-associated lung disease can have either obstructive 31 or restrictive 32 respiratory phenotypes, and we similarly found pulmonary function testing to be variable among our patients. In contrast, diminished DLCO was a more consistent finding that was noted in 4 of the 5 patients tested.…”

British Thoracic Society guideline for non-CFbronchiectasis

“…Significant antibody deficiency confers a particular susceptibility to mucosal infection with encapsulated organisms. Recurrent upper and lower respiratory tract infections with Streptococcus pneumoniae , Haemophilus influenzae (frequently untypable) and Moraxella catarrhalis are characteristic of antibody deficiency in both children and adults 63 94 96 114 117 119–121. This is seen before diagnosis/institution of therapy but also as breakthrough episodes in patients receiving immunoglobulin replacement therapy 485…”

Common Variable Immunodeficiency: Etiological and Treatment Issues

“…While encapsulated organisms such as Streptococcus pneumoniae or atypicals such as Mycobacteria are the most commonly identified pathogens, patients may also present with infections typically associated with T cell defects such as Pneumocystis jiroveci [14,19]. As might be expected, it has long been recognized that chronic inflammation and recurrent infections associated with CVID may result in severe bronchiectasis and pulmonary fibrosis [19,35,38,39,40,41,42,43,44,45,46,47,48], possibly influenced by mannose-binding lectin polymorphisms [42]. Further, patients with CVID are at risk for granulomatous infiltration and interstitial pneumonia in a pattern termed GLILD (granulomatous/lymphocytic interstitial lung disease) [19,44,48,49,50,51].…”