Primary Immunodeficiencies: Epidemiology in the Maghreb

Area of Science:

• Immunology
• Genetics
• Public Health

Background:

• Primary Immunodeficiency (PID) comprises 330 rare hereditary disorders increasing susceptibility to infections, allergies, autoimmunity, and neoplasia.
• North American PID registries report higher prevalence rates compared to Maghreb registries, despite high consanguinity in the latter.
• Understanding PID prevalence is crucial for public health initiatives and patient care.

Purpose of the Study:

• To compare the prevalence and registry coverage rates of Primary Immunodeficiency (PID) in the Maghreb region with estimates derived from the USA.
• To evaluate the impact of high consanguinity rates on PID prevalence in the Maghreb.
• To assess the current data collection efficiency of PID registries in the Maghreb.

Main Methods:

• Systematic search of Maghreb PID registries to determine current patient numbers.
• Estimation of expected PID prevalence based on recent international publications, particularly from the USA.
• Calculation of the coverage rate of Maghreb registries against estimated values and analysis of consanguinity's influence.

Main Results:

• The Maghreb PID prevalence is estimated at 2.56 per 100,000 inhabitants, with Tunisia showing the highest rate (8.70).
• Extrapolating USA prevalence data suggests 27,588 PID patients in the Maghreb, indicating a low registry coverage rate of 8.90%.
• Maghreb's PID prevalence aligns with the Arab world's average (2.04/100,000), but the number of diagnosed patients is significantly lower than expected.

Conclusions:

• The number of diagnosed Primary Immunodeficiency (PID) patients in the Maghreb is substantially lower than global estimates, highlighting underdiagnosis and low registry coverage.
• High consanguinity rates in the Maghreb region may contribute to the genetic burden of PID, necessitating focused research and intervention.
• Urgent attention from governments and research bodies in the Maghreb is required to improve PID diagnosis, registry completeness, and patient management.