What is the morbidity and mortality associated with common variable immunodeficiency (CVID)?

Updated: Oct 16, 2018
  • Author: C Lucy Park, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

The prognosis for patients with common variable immunodeficiency is reasonably good if they do not have bronchiectasis and chronic lung damage, severe autoimmune disease, or malignancy.

Resnick et al summarized morbidity and mortality among 473 patients with common variable immunodeficiency at Mt. Sinai Hospital, NY over the past 4 decades. [5] Reduced survival was associated with age at diagnosis, lower baseline IgG, higher IgM, and fewer peripheral B cells. The risk of death was 11 times higher for patients with noninfectious complications. Mortality was associated with lymphoma, any form of hepatitis, functional or structural lung impairment, and GI disease, but not with bronchiectasis, autoimmunity, other cancers, or granulomatous disease.

Chapel et al reported European common variable immunodeficiency registry data that included 326 patients followed for at least 10 years since onset of symptoms. [6] The 75th percentile for survival was 25 years after diagnosis, and the 60th percentile for survival was 41 years after diagnosis. No associations between survival and sex or initial serum IgG, IgA, or IgM levels were noted. In the European registry, the highest mortality rates were in patients with the enteropathy phenotype or the polyclonal lymphocytic infiltrative phenotype. An association between increased mortality and lymphoid malignancy was also noted.


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