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Chest Complications in Patients with Primary Antibody Deficiency Syndromes (PADS)

  • Helen M. ChapelEmail author
  • Consuelo Anzilotti
  • Smita Y. Patel
Chapter
Part of the Rare Diseases of the Immune System book series (RDIS)

Abstract

Symptomatic primary antibody deficiency syndromes (PADS) range from severe forms, such as common variable immunodeficiency disorders and X-linked agammaglobulinemia, to partial antibody deficiencies. All patients with failure of antibody production suffer from chest infections with common organisms, which should prompt diagnosis. Patients with proven antibody failure require replacement immunoglobulin therapy.

Chest infections are treated with appropriate antibiotics early and for longer in antibody failure than in immunocompetent individuals to prevent structural lung damage, since bronchiectasis is a common feature in patients with severe PADS. Bronchiectasis necessitates breakthrough infection documentation as well as CT monitoring for progression, which is not inevitable in patients receiving adequate doses of replacement immunoglobulin.

Interstitial lung disease is a particular feature in about 15% or so of sporadic CVID patients, though this may represent several different conditions depending on aetiology, and prognosis is uncertain. Biopsy is essential to distinguish lymphoid malignancy and to determine appropriate therapeutic interventions. Until there is a standard protocol for reporting CT findings, and an internationally agreed histological classification for biopsies that can predict prognosis, this complication remains difficult to investigate and treat successfully.

Keywords

Common variable immunodeficiency disorders Interstitial lung disease Bronchiectasis Chest infections Replacement immunoglobulin Lymphoproliferation Clinical phenotype 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Helen M. Chapel
    • 1
    Email author
  • Consuelo Anzilotti
    • 1
  • Smita Y. Patel
    • 1
  1. 1.Primary Immunodeficiency Unit, Experimental Medicine, Nuffield Department of MedicineJohn Radcliffe Hospital, University of OxfordOxfordUK

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