Agammaglobulinemia and respiratory complications

Reference :Fekrvand, S, Yazdani, R, Olbrich, P, et al. Evaluation of respiratory complications in patients with X‐linked and autosomal recessive agammaglobulinemia. Pediatr Allergy Immunol. 2020; 31: 405– 417.

Journal: Pediatric Allergy and Immunology

Summary: 1. 115 patients from 1983-2018 with B cells <2%, low IgG levels and recurrent infections were included and Pulmonary function tests (PFT) and HRCT in patients on follow-up

2. Pneumonia was commonest respiratory complication and these patients had low CD4 counts

3. On HRCT, around 45% patients had bronchiectasis which was associated with very low IgG levels , 20.7% patients had mild pulmonary involvement 

4. Patients with low FVC, FEV1, and FEV1/FVC values had lower Bhalla scores and increased frequency of bronchiectasis

Bottom line: 1. Respiratory tract evaluation should be done routinely in these patients due to increased incidence of respiratory tract infections improving the quality of life and prognosis

                        2. PFT can be used intermittently to monitor the same and HRCT be reserved for patients with delayed diagnosis and strong suspicion of lung parenchymal damage due to increased susceptibility to radiation

lungs, branches, tree