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Which are the patients with CVID who die too early?

Published online March 14, 2025

Common variable immunodeficiency (CVID) is the most common primary symptomatic immunodeficiency in adults. Patients with CVID have low immunoglobulins and poor vaccine responses. Patients may only present with infections (infection-only CVID, CVIDio) or also with immune-mediated complications such as lymphoproliferation, autoimmunity, inflammatory organ disease, and malignancy (complicated CVID, CVIDc). Immunoglobulin replacement therapy does not control immune-mediated complications. CVIDc patients present reduced overall survival compared to CVIDio and often die prematurely. The leading causes of death comprise of malignancies and infections. To date, there is a paucity of data on the prognostic value of specific complications and laboratory parameters associated with premature mortality, and a prognostic score is urgently needed to anticipate a poor outcome and tailor a personalized management for patients with CVIDc in time. 

An article by Bez et al., recently published in The Journal of Allergy and Clinical Immunology: In Practice, reports a monocentric study aimed at (1) describing the characteristics of deceased patients with CVID at a tertiary referral center (CCI, Freiburg, Germany) and (2) investigating potential clinical and  laboratory parameters associated with the premature mortality in a group of CVIDc patients and a matched control group not only at the time of death but also three and five years before. The exploratory case-match study used a convenience sample of 37 cases who died prematurely (i.e. younger than 70 years), excluding those who died from transplant-related and non-health- related causes. For each case, two controls were identified matched for sex and birth year ± 5 years. With one control missing, 73 matched controls were included in the final analysis. The age of death of case was chosen as the age of matching (AOM). Clinical and laboratory characteristics of the cases and controls were analyzed at the nearest available visit at all three time points. The best candidate parameters were compared by a multivariable conditional logistic regression analysis. 

The study included 497 patients, of whom 57 (11.5%) died. The poor prognosis of CVIDc compared to CVIDio was confirmed. The most common causes of death among CVIDc patients were infections and neoplasia but with an altered infection profile when compared to the typical profile in CVID. The exploratory case-control study demonstrated for the first time that cases had a higher prevalence of severe enteropathy, severe interstitial lung disease, and hepatopathy than controls already at three years before death. This was associated with a higher frequency of lymphopenia, thrombocytopenia, and elevated liver enzymes. The identified clinical and laboratory parameters need to be confirmed in a prospective multicenter study in order to establish a robust prospective score that will allow better counseling of affected patients. 

The Journal of Allergy and Clinical Immunology: In Practice is an official journal of the AAAAI, focusing on practical information for the practicing clinician.

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