Pulmonary Manifestations of Common Variable Immunodeficiency. Issue 6 (November 2018)

• Record Type: Journal Article
• Authors: Bang, Tami J.; Richards, John Caleb; Olson, Amy L.; Groshong, Steve D.; Gelfand, Erwin W.; Lynch, David A.
• Is Part Of: Journal of thoracic imaging. Volume 33:Issue 6(2018)
• Publication Date: 2018-11
• Subjects: common variable immunodeficiency -- CVID -- granulomatous lymphocytic interstitial lung disease -- GLILD -- Kabuki syndrome; Chest -- Radiography -- Periodicals; Chest -- Diseases -- Diagnosis -- Periodicals; 617.540757
• DOI: 10.1097/RTI.0000000000000350
• Languages: English
• ISSNs: 0883-5993

Abstract

Abstract : Common variable immunodeficiency (CVID) is associated with significant chronic lung disease. The purpose of this paper was to describe the clinical, radiologic, and pathologic findings of CVID-associated lung diseases. These include airways' disease, interstitial lung disease, lymphoma, and mucosa-associated lymphoid tissue lymphoma. In addition, a genetic syndrome termed Kabuki syndrome results in CVID-like immune abnormalities. These patients may also present with CVID-associated lung disease. Awareness and precise identification of CVID-associated lung disease may allow for better assessment of prognosis and direction of therapy.