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Management of Europeans with CVID varied across region

The management of patients with common variable immunodeficiency may vary across Europe, affecting various outcome measures, according to data from a recently published study.

“Common variable immunodeficiency (CVID) forms a heterogeneous group of disorders characterized by impaired antibody provision. It is the most frequent clinically symptomatic primary antibody disorder, with a prevalence of approximately 1:50,000 to 1:25,000,” researchers wrote.

The main features of the immunodeficiency include respiratory tract infections and their associated complications, enteropathy, autoimmunity, and lymphoproliferative disorders.

Researchers retrospectively examined the European Society for Immunodeficiencies registry for data on 2,212 patients with CVID from 28 medical centers. They found that early disease onset (<10 years) occurred frequently in the cohort (33.7%), particularly among men (39.8%), who were more likely to develop pneumonia and less likely to develop other complications, suggesting a disease entity.

Delayed diagnosis ranged between 4 to 5 years in many countries, and was found to be particularly high in patients with early-onset CVID. Researchers also observed that a 1-year increase in age at diagnosis was associated with a 4.5% increase in the risk for death.

“We found that age at diagnosis and age at onset were both significantly associated with patient survival (P<.001),” the researchers wrote. “Older age at onset and older age at diagnosis were both associated with an increased risk of death at any time.”

Differences in immunoglobulin dosing ranged from 130 mg/kg monthly to 750 mg/kg monthly. Patients with low trough levels of less than 4 g/L displayed poor clinical outcomes, while those with higher trough levels demonstrated a reduced incidence of serious bacterial infections.

“We have shown that using an electronic patient registry to acquire data on patients with CVID from a large number of medical centers is one possibility to answer disease-specific questions,” researchers wrote.

Disclosure: See the study for a full list of relevant financial disclosures.