Evaluation of the frequency and diagnostic delay of primary immunodeficiency disorders among suspected patients based on the 10 warning sign criteria: A cross-sectional study in Iran

Abstract

Introduction: The prevalence of undiagnosed primary immunodeficiency diseases is remarkably high and contributes to increasing the rate of morbidity and mortality among this group of patients.

Objective: To examine the 10 warning sign scoring system in patients suspected of primary immune deficiency and also estimate the diagnostic delay in patients with proven disease.

Methods: This descriptive cross-sectional study was carried out during the years 2015-2016 in Ali Asghar (AS) Clinic and Hospital. Two hundred patients with suspected primary immune deficiency disease were eligible for inclusion in the study. Multivariable logistic regression analysis was used to determine the relation between findings.

Results: In this study, the majority of suspected cases of immunodeficiency were males (57%) with a mean age of 3.33±2.89 years. Twenty-one (10.5%) patients were diagnosed with immunodeficiency disease. The mean diagnostic delay among primary immune deficient patients was 2.05±1.7 years. There was a significant relationship between having parental consanguinity (OR=2.68, 95% CI: 1.07-6.70), allergies (OR=5.03, 95% CI: 1.13-22.31), vaccine adverse effects (OR=9.31, 95% CI: 1.24-69.96) and primary immune deficiency diagnosis. No association was observed between age (OR=0.98, 95% CI: 0.84-1.14), gender (OR=0.99, 95% CI: 0.39-2.47), immune deficiency scoring (OR=0.68, 95% CI: 0.31-1.45) and primary immune deficiency diagnosis.

Conclusion: Ten warning sign scoring system is of less value to consider a patient suspected of having primary immune deficiency. There is a meaningful delay in diagnosis of primary immune deficiencies especially in antibody deficiency defects which seeks further upgrading of knowledge in physicians.

Keywords: Common variable immune deficiency; Hypogammaglobulinemia; Immune deficiency disease; Severe combined immune deficiency.