Pulmonary Pathology in Agammaglobulinemia: Diagnosis and Treatment

Airway infections are the most common complication in patients with agammaglobulinemia. A vicious cycle of infection, inflammation, increased mucus production, and impaired airway clearance leads to structural airway damage, in particular to bronchiectasis. This chapter discusses the diagnostic and therapeutic approach to the patient with agammaglobulinemia with lung disease. The discussion refers to the available evidence with XLA patients and to what can be learned from other patient groups with chronic lung disease, such as cystic fibrosis or non-CF bronchiectasis.