Abstract
Bronchiectasis, the presence of bronchial wall thickening with airway dilatation, is a particularly challenging complication of primary antibody deficiencies. While susceptibility to infections may be the primary factor leading to the development of bronchiectasis in these patients, the condition may develop in the absence of known infections. Once bronchiectasis is present, the lungs are subject to a progressive cycle involving both infectious and non-infectious factors. If bronchiectasis is not identified or not managed appropriately, the cycle proceeds unchecked and yields advanced and permanent lung damage. Severe symptoms may limit exercise tolerance, require frequent hospitalizations, profoundly impair quality of life (QOL), and lead to early death. This review article focuses on the appropriate identification and management of bronchiectasis in patients with primary antibody deficiencies. The underlying immune deficiency and the bronchiectasis need to be treated from combined immunology and pulmonary perspectives, reflected in this review by experts from both fields. An aggressive multidisciplinary approach may reduce exacerbations and slow the progression of permanent lung damage.
Highlights
Primary antibody deficiencies are the most common inherited form of primary immunodeficiency [1]
Cell-mediated immune response to non-typeable Haemophilus influenzae (NTHi), the most common respiratory pathogen in patients with bronchiectasis, has been found to be impaired in children with chronic suppurative lung disease (CSLD), which is considered a prelude to bronchiectasis
Patients with agammaglobulinemia and common variable immune deficiency (CVID) may still develop chronic lung disease [59] and may need prolonged or prophylactic antibiotics in order to place a halt on the recurrent lung infections
Summary
Primary antibody deficiencies are the most common inherited form of primary immunodeficiency [1]. Studies of bronchiectasis in general, summarized below, have demonstrated that a disordered inflammatory response is an important variable, as is an impaired T cell response to pathogens These are problems which are relatively common in some forms of antibody deficiencies such as CVID. Cell-mediated immune response to NTHi, the most common respiratory pathogen in patients with bronchiectasis, has been found to be impaired in children with chronic suppurative lung disease (CSLD), which is considered a prelude to bronchiectasis. In a study of adults with bronchiectasis, cytotoxic T cell demonstrated less IFN-gamma production in vitro to NTHi compared to controls [29] This evidence suggests that an impaired cellmediated immune response may lead to increased susceptibility to NTHi and contribute to the pathogenesis of CSLD, the precursor of bronchiectasis. Understanding how underlying systemic immune dysregulation contributes to the development of bronchiectasis in patients with antibody deficiencies holds important potential implications for both prognosis and treatment
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