The aim of this exhibit is to illustrate the thoracic imaging findings in adult patients with common variable immunodeficiency (CVID).
Common variable immunodeficiency (CVID) is a primary humoral immunodeficiency disorder,characterized by impaired B cell differentiation and deficiencies in some or all classes of immunoglobulins. It is not a diagnosis in itself but rather incorporates several disorders, characterized by impaired antibody production.
Diagnostic criteria for CVID are as follows:
≥2 standard deviations (SD) below mean for age in serum IgG & IgA and
onset of immunodeficiency >2 years of age and
absent isohemagglutinins and/or poor response to vaccinations and
no other cause of hypogammaglobulinemia...
Findings and procedure details
There is considerable overlap in imaging findings between acute and chronic, infectious and non-infections complications in patients with CVID.
Infection is the most important clinical problem in patients with CVID both at diagnosis and during follow-up.3
The imaging findings of lower respiratory tract infection in patients with CVID are similar to those in immunocompetent patients. Infection can appear as tree-in-bud nodularity when the bronchioles are affected, consolidation (either segmental or lobar) when the alveoli are affected and/or as pulmonary abscess. Linear parenchymal scarring...
Imaging plays an important role in the diagnosis and quantification ofthoracic disease in patients with CVID, in directing treatment and in monitoring response to same. Granulomatous disease should be considered in all patients with CVID who have pulmonary nodules, thoracic lymphadenopathy and/or splenomegaly on CT.
Personal information and conflict of interest
C. Ní Leidhin; Dublin/IE - nothing to disclose M. L. Bambrick; Dublin/IE - nothing to disclose R. M. Heaney; Dublin/IE - nothing to disclose P. Beddy; Dublin/IE - nothing to disclose N. Conlon; Dublin/IE - nothing to disclose
1.Conley ME, Notarangelo LD and Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999;93:190-7.
2.Gathmann B, Mahlaoui N, Ceredih LG et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol. 2014;134:116-26.
3.Quinti I, Soresina A, Spadaro G et al. Long-term follow-up and outcome of a large cohort of patients with common variable immunodeficiency. J Clin Immunol. 2007;27:308-16.
4.Wehr C, Kivioja T, Schmitt C et al....