Cytomegalovirus in primary immunodeficiency.

Cytomegalovirus (CMV) infection and disease are well described in the setting of secondary immunodeficiency. Less is known about CMV in the context of primary immunodeficiencies (PIDs), where inborn errors in one or more arms of the immune system result in variable degrees of CMV susceptibility. PID presents unique challenges in the diagnosis and management of CMV disease. The clinical presentation of CMV in PID is often severe, accelerated by underlying immune dysregulation and iatrogenic immunosuppression. Here we describe the clinical significance of CMV infection in PID, the key components of immune defence against CMV and how these are affected in specific PIDs. CMV disease is under-recognized as a complication of common variable immunodeficiency (CVID). High rates of CMV end-organ disease, mortality, development of CMV resistance and prolonged antiviral use have been observed in individuals with CVID. We recommend that clinicians tailor their approach to the individual based on their underlying immune deficit and maintain a high index of suspicion and low threshold for treatment. More research is required to improve stratification of CMV risk in PID, develop new diagnostic tools and manage end-organ disease in this cohort. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.

Citation

Jack Godsell, Samantha Chan, Charlotte Slade, Vanessa Bryant, Jo Anne Douglass, Joe Sasadeusz, Michelle K Yong. Cytomegalovirus in primary immunodeficiency. Current opinion in infectious diseases. 2021 Dec 01;34(6):663-671

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PMID: 34608876

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