Primary immunodeficiencies (PID) are rare diseases, often due to a genetic defect, affecting pediatric and adult patients. They are characterized by poor and dysregulated function <span>of the immune system leading to recurrent infections and to other non-infectious immune-mediated manifestations. Atopic diseases like asthma, atopic dermatitis, chronic rhinosinusitis with nasal polyps are much more frequent, and may also affect PID patients. In these diseases, the immune system is mainly working in “Th2 mode”. Over the last few years, biological drugs targeting the mechanisms of atopic diseases (named “Th2 biologicals”) have been increasingly used, with positive impact on disease control, improvement in quality of life and good tolerance. Since these drugs act by limiting the function of the immune system, their use in PID patients may raise concerns regarding a possible increase in the risk of infections. Moreover, their specific effect in humans whose immune system is working less and differently, if compared to healthy people, is still unknown.This project will explore, through a specifically designed survey, the use in real life of “Th2 biologicals” in PID patients across European atopic diseases outpatient clinics. All collected data will be analyzed to better understand the effect of these widespread used drugs in such rare conditions and to provide recommendations on their efficacy and safety, that will help physicians and PID patients to face atopic manifestations.
WG Immunodeficiencies (Basic & Clinical Immunology Section)
Chair: Francesco Cinetto
Secretary: Maria Escribese