Published online: September 30, 2016
Primary immunodeficiencies (PIDs) induce higher susceptibility to recurrent infections, autoimmunity, allergy, cancer. Many patients require life-long therapy and most children now reach adulthood, but little is known of the health condition in this young population.
In a study recently published in The Journal of Allergy & Clinical Immunology (JACI), Barlogis and colleagues conducted a multicenter prospective follow-up program addressing the physical health condition of French patients with PIDs who reached adulthood and the impact on their quality of life, using both a health status questionnaire and a quality of life questionnaire
Among the 889 participants, 329 were adults with a mean age at study participation of 27 years and a mean age at PID diagnosis of 6 years. Of these adults, 58 had undergone transplantation. In terms of health condition, a severity score was assigned to each condition: grade 1, mild; grade 2, moderate; grade 3, severe; and grade 4, life-threatening/disabling. The authors show that the prevalence of severe or life-threatening health conditions is very high in participants, particularly concerning surgery, respiratory, gastrointestinal, ENT, and dermatological conditions. Only fewer than 15% of adults with PIDs had never experienced severe (grade3) or life-threatening (grade 4) conditions. Above all, 7.6% of the patients reported a malignancy which was markedly high considering the young age of our population. In terms of quality of life, adults with PID scored significantly lower for all domains of quality of life when compared with French population normal values. Interestingly, Quality of life was affected neither by the PID diagnosis nor the duration of the disease nor the current prophylactic therapy. However, when patients had received transplantation they scored significantly higher in the physical composite score and the general health subscale. Lastly, the authors showed that quality of life was strongly associated with the burden of health conditions.
The authors’ findings highlight that this young population suffering from several health conditions has a significantly compromised quality of life, which emphasize the need to closely monitor this vulnerable population.
The Journal of Allergy and Clinical Immunology (JACI) is an official scientific journal of the AAAAI, and is the most-cited journal in the field of allergy and clinical immunology.