Journal of Clinical Immunology

, Volume 38, Issue 3, pp 237–246 | Cite as

Immunologist’s Perspectives on Assessment and Management of Lung Disease in CVID: a Survey of the Membership of the Clinical Immunology Society and the European Society for Immunodeficiencies

  • Javeed Akhter
  • Cheryl A. Lefaiver
  • Christopher Scalchunes
  • Michael DiGirolamo
  • Klaus Warnatz
Letter to Editor


Over 350 primary immunodeficiency diseases are currently recognized. Other than selective IgA deficiency, common variable immunodeficiency (CVID) is the most common primary immunodeficiency disease [1]. The prevalence of CVID is estimated to be one in 25,000, but this is at best an educated guess. An International Consensus Document (ICON) [2] has proposed a revised definition for CVID and compared it with the previously published criteria suggested by Ameratunga et al. [3]. The usual practice is to use simpler criteria proposed by the European Society of Immune Deficiency Disorders (ESID) and Pan-American Group for Immunodeficiency (PAGID) [4].

Recurrent bacterial infections are a hallmark of CVID, the most common of which are sino-pulmonary infections. These may result in a chronic and progressive bronchiectasis. Patients with CVID are also prone to developing interstitial lung disease (ILD) (some with non-caseating granulomas) or ILD, and bronchiectasis may be present...


Compliance with Ethical Standards

Conflicts of Interest

None of the authors received money or other reimbursement for their work on this project or have conflicts of interest.


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Javeed Akhter
    • 1
  • Cheryl A. Lefaiver
    • 1
  • Christopher Scalchunes
    • 2
  • Michael DiGirolamo
    • 1
  • Klaus Warnatz
    • 3
  1. 1.Advocate Children’s HospitalOak LawnUSA
  2. 2.Immune Deficiency FoundationTowsonUSA
  3. 3.Centre for Chronic ImmunodeficiencyFreiburgGermany

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