Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study

Vincent Barlogis; Nizar Mahlaoui; Pascal Auquier; Isabelle Pellier; Fanny Fouyssac; Camille Vercasson; Maya Allouche; Carolina Brito De Azevedo; Felipe Suarez; Despina Moshous; Bénédicte Neven; Marlène Pasquet; Eric Jeziorski; Nathalie Aladjidi; Nicolas Schleinitz; Caroline Thomas; Virginie Gandemer; Françoise Mazingue; Patrick Lutz; Olivier Hermine; Capucine Picard; Stéphane Blanche; Gérard Michel; Alain Fischer

doi:10.1016/j.jaci.2016.08.027

Physical health conditions and quality of life in adults with primary immunodeficiency diagnosed during childhood: A French Reference Center for PIDs (CEREDIH) study

J Allergy Clin Immunol. 2017 Apr;139(4):1275-1281.e7. doi: 10.1016/j.jaci.2016.08.027. Epub 2016 Sep 30.

Authors

Vincent Barlogis¹, Nizar Mahlaoui², Pascal Auquier³, Isabelle Pellier⁴, Fanny Fouyssac⁵, Camille Vercasson³, Maya Allouche⁶, Carolina Brito De Azevedo⁷, Felipe Suarez⁸, Despina Moshous², Bénédicte Neven², Marlène Pasquet⁹, Eric Jeziorski¹⁰, Nathalie Aladjidi¹¹, Nicolas Schleinitz¹², Caroline Thomas¹³, Virginie Gandemer¹⁴, Françoise Mazingue¹⁵, Patrick Lutz¹⁶, Olivier Hermine¹⁷, Capucine Picard², Stéphane Blanche², Gérard Michel¹⁸, Alain Fischer¹⁹

Affiliations

¹ Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France; French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Public Health-EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France. Electronic address: vincent.barlogis@ap-hm.fr.
² French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
³ Department of Public Health-EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France.
⁴ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Oncohematology, University Hospital of Angers, Angers, France.
⁵ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Hematology-Oncology, University Hospital of Nancy, Nancy, France.
⁶ Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France.
⁷ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
⁸ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Adult Hematology Unit, Necker University Hospital, APHP, University Paris Descartes, Paris, France; CNRS ERL8254, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France.
⁹ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Département d'Hématologie, Centre Hospitalier Universitaire Toulouse Purpan and INSERM, CRCT, IUCT-Oncopole, Toulouse, France.
¹⁰ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Service de Pédiatrie Générale et Infectiologie et Immunologie Clinique, Hôpital Arnaud de Villeneuve, Montpellier University Hospital, Montpellier, France.
¹¹ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Hematology and CIC 0005, INSERM CICP, University Hospital of Bordeaux, Bordeaux, France.
¹² French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Internal Medicine, CHU Timone, Assistance Publique-Hôpitaux de Marseille, Marseille, France.
¹³ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Hematology-Oncology Unit, Hôpital Mère Enfant, University Hospital of Nantes, Nantes, France.
¹⁴ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatric Hematology/Oncology, University Hospital of Rennes, Rennes, France.
¹⁵ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Pediatrics, Hôpital Jeanne de Flandre, University Hospital of Lille, Lille, France.
¹⁶ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Hematology-Oncology, EA3430, Strasbourg, France.
¹⁷ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Adult Hematology Unit, Necker University Hospital, APHP, University Paris Descartes, Paris, France.
¹⁸ Department of Pediatric Hematology-Oncology, Assistance Publique-Hôpitaux de Marseille, Marseille, France; French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Department of Public Health-EA 3279 Research Unit, University Hospital Marseille, Aix-Marseille University, Marseille, France.
¹⁹ French National Reference Center for Primary Immune Deficiency (CEREDIH), Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Pediatric Immuno-Haematology and Rheumatology Unit, Necker Enfants Malades University Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM UMR1163, Imagine Institute, Necker Medical School, Sorbonne Paris Cité, University Paris Descartes, Paris, France; Collège de France, Paris, France.

PMID: 27697497
DOI: 10.1016/j.jaci.2016.08.027

Abstract

Background: Most children with primary immunodeficiencies (PIDs) now reach adulthood. However, few studies have evaluated their health status and health-related quality of life (HRQoL).

Objective: To investigate long-term morbidity, the French Reference Center for PIDs initiated a prospective multicenter cohort: the French Childhood Immune Deficiency Long-term Cohort. The data collected were used to assess the physical health condition of patients who reached adulthood and the effect on their quality of life.

Methods: Patients were asked to complete health status questionnaires. A severity score (grade 1 [mild] to grade 4 [life-threatening]) was assigned to each health condition. The HRQoL of patients was compared with age- and sex-matched French normal values by using the 36-item Short-Form Survey (SF-36) HRQoL questionnaire.

Results: Among 329 participants, the mean age at evaluation was 27.6 years, with a 21-year mean follow-up after diagnosis; 43% reported at least 1 grade 4 health condition, and 86% reported at least 1 grade 3 (severe) or 4 health condition. Twenty-five (7.6%) patients had been treated for cancer. Compared with the French normal values, adults with PIDs scored significantly lower for all HRQoL domains. HRQoL was strongly associated with the burden of health conditions. The association with grade 4 or grade 3-4 health conditions was highly significant for all physical and mental domains.

Conclusion: Adults with PIDs diagnosed during childhood experienced a heavy burden of health conditions, which affected their HRQoL. Our results emphasize the need to closely monitor this vulnerable population.

Keywords: Primary immunodeficiency; childhood; health status; quality of life; young adults.

Publication types

Multicenter Study

MeSH terms

Adult
Age of Onset
Child
Cohort Studies
Female
France
Health Status*
Humans
Immunologic Deficiency Syndromes / complications*
Male
Quality of Life*
Surveys and Questionnaires
Time