Health policy for common variable immunodeficiency: burden of the disease.

BACKGROUND

Common variable immunodeficiency (CVID) is a primary immunodeficiency disease characterized by recurrent infections and increased susceptibility to autoimmunity and malignancy.

OBJECTIVES

This study was performed to estimate the burden of CVID in Iran during 1985-2008 based on incidence, mortality, and disability-adjusted life-years (DALY).

METHODS

The methods developed by the World Health Organization for national burden of disease studies were applied to estimate the incidence of disease and thus calculate the years of life lost due to premature mortality (YLL), years living with disability (YLD), and DALYs.

RESULTS

The average age-adjusted incidence of CVID was 1 case per 200 000 per year; the average age-adjusted prevalence was 1 case per 91 000 per year. The burden of CVID (DALYs) was 25.21 years per 100000 individuals (17.86 for YLL and 7.35 for YLD). DALYs increased significantly in patients aged 5-14 years and in those with polyclonal lymphocytic infiltration phenotypes (P < .001).

CONCLUSIONS

Based on the measurement of DALY in patients with CVID, reducing the rate of premature death in the polyclonal lymphocytic infiltration phenotype and the rate of infectious episodes in patients with the infectious only phenotype and appropriate management with regular intravenous immunoglobulin represent the best approach to decreasing the burden of CVID.