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Pulmonary Pathology in Agammaglobulinemia: Diagnosis and Treatment

  • Chapter
Agammaglobulinemia

Part of the book series: Rare Diseases of the Immune System ((RDIS,volume 4))

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Abstract

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.

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  1. Paediatric Pulmonology, Allergy and Neonatology, Hanover Medical School, Hannover, Germany

    Ulrich Baumann

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  1. Pediatrics Clinic, Department of Clinical and Experimental Sciences, University of Brescia and Spedali Civili of Brescia, Brescia, Italy

    Alessandro Plebani

  2. Pediatrics Clinic, Department of Clinical and Experimental Sciences, University of Brescia and Spedali Civili of Brescia, Brescia, Italy

    Vassilios Lougaris

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Baumann, U. (2015). Pulmonary Pathology in Agammaglobulinemia: Diagnosis and Treatment. In: Plebani, A., Lougaris, V. (eds) Agammaglobulinemia. Rare Diseases of the Immune System, vol 4. Springer, Cham. https://doi.org/10.1007/978-3-319-22714-6_3

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