Affiliations 

  • 1 Pediatric Allergy, Immunology and Rheumatology Unit, Children's Hospital, Ain Shams University, Cairo, Egypt
  • 2 Clínica de Alergia Pediátrica- Mexico
  • 3 Allergy and Clinical Immunology, Hospital Nacional Edgardo Rebagliati Martins, Peru
  • 4 Allergy and Clinical Immunology Unit, Pediatric Department, AlSabah Hospital, Faculty of Medicine, Kuwait University, Kuwait
  • 5 Department of Pediatrics, Pulmonary Division- Allergy and Immunology Section, Qatar
  • 6 Department of Immunology, Institute of Biomedical Sciences, University of Sao Paulo, Sao Paulo, Brazil
  • 7 Department of Allergy and Immunology- Royal Children's Hospital, Australia
  • 8 Paediatric Infectious Diseases Unit, Red Cross War Memorial Children's Hospital and the Department of Paediatrics and Child Health, University of Cape Town, South Africa
  • 9 Allergy and Immunology Unit- Soba University Hospital and Faculty of Medicine, University of Khartoum, Sudan
  • 10 Instituto Nacional de Pediatría, Immunodeficiencies Laboratory, Mexico
  • 11 Immunoallergy Department, Coimbra Hospital and University Centre (CHUC), Portugal
  • 12 Department of Pediatrics, Federal University of Parana, Brazil
  • 13 Pediatric Allergy, Immunology and Rheumatology, Columbia University Medical Center, New York, USA
  • 14 Department of Pediatrics- Division of Immunology, Cairo University, Egypt
  • 15 National Human Genome Research Institute, National Institute of Health, USA
  • 16 Department of Allergy and Clinical Immunology- Children's Hospital of Wisconsin, Medical College of Wisconsin, USA
  • 17 Department of Pediatrics-Tokyo Medical and Dental University, Japan
  • 18 Department of Internal Medicine and Clinical Immunology, Hotel Dieu de France Hospital, Beirut, Lebanon
  • 19 Department of Immunology, Pasteur Institute of Algeria, Algeria
  • 20 Department of Immunology, University Hospital Motol and the 2nd Faculty of Medicine, Charles University, Czech Republic
  • 21 Division of Pediatric Allergy, University of Cape Town, South Africa
  • 22 Department of Pediatrics, Immuno-hematology and Stem Cell Transplantation- Bone Marrow Transplantation Center Tunisia
  • 23 Department of Medicine and Sciences of Aging, University "G. D'Annunzio", Chieti-Pescara - and YDA, Institute of Clinical Immunotherapy and Advanced Biological Treatments, Pescara, Italy
  • 24 Translational Medical Sciences-Pediatric Immunology, Italy
  • 25 Department of Pediatrics and Child Health- Aga Khan University Hospital, Karachi, Pakistan
  • 26 Department of Molecular Medicine, PID Reference Centre, Sapienza University of Rome, Italy
  • 27 Department of Pediatric Hematology Oncology, Super Specialty Pediatric Hospital and Post Graduate Teaching Institute Noida, Delhi NCR 201303, India
  • 28 Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran
  • 29 Department of Pediatrics and Chief, Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India
  • 30 Hospital Tunku Azizah, Women and Children Hospital Kuala Lumpur, Malaysia
  • 31 Gaafer Ibn Ouf Specialist Paediatric Hospital, Sudan
  • 32 Department of Pulmonology, Division of Allergy, University Hospital of Montpellier, Montpellier, France
  • 33 Department of Pediatric Infectious Diseases and Immunology- Shupyk National Medical Academy of Postgraduate Education, Ukraine
  • 34 Department of Pediatrics, Children's Hospital of Philadelphia, USA
World Allergy Organ J, 2022 Jun;15(6):100657.
PMID: 35783543 DOI: 10.1016/j.waojou.2022.100657

Abstract

BACKGROUND: Allergies have long been observed in Inborn Errors of Immunity (IEI) and might even be the first presentation resulting in delayed diagnosis or misdiagnosis in some cases. However, data on the prevalence of allergic diseases among IEI patients are limited and contradictory.

OBJECTIVE: To provide a worldwide view of allergic diseases, across a broad spectrum of IEI, and their impact on the timely diagnosis of IEI.

METHODS: This is a worldwide study, conceived by the World Allergy Organization (WAO) Inborn Errors of Immunity Committee. A questionnaire was developed and pilot-tested and was sent via email to collect data from 61 immunology centers known to treat pediatric and/or adult IEI patients in 41 countries. In addition, a query was submitted to The United States Immunodeficiency Network (USIDNET) at its website.

RESULTS: Thirty centers in 23 countries caring for a total 8450 IEI patients responded. The USIDNET dataset included 2332 patients. Data from responders showed that a median (IQR) of 16.3% (10-28.8%) of patients experienced allergic diseases during the course of their IEI as follows: 3.6% (1.3-11.3%) had bronchial asthma, 3.6% (1.9-9.1%) atopic dermatitis, 3.0% (1.0-7.8%) allergic rhinitis, and 1.3% (0.5-3.3%) food allergy. As per the USIDNET data, the frequency of allergy among IEI patients was 68.8% (bronchial asthma in 46.9%). The percentage of IEI patients who presented initially with allergic disorders was 8% (5-25%) and diagnosis delay was reported in 7.5% (0.9-20.6%). Predominantly antibody deficiencies had the highest frequency of allergic disease followed by combined immunodeficiency with a frequency of 40.3% (19.2-62.5%) and 20.0% (10-32%) respectively. As per the data of centers, anaphylaxis occurred in 25/8450 patients (0.3%) whereas per USIDNET dataset, it occurred in 249/2332 (10.6%); drugs and food allergy were the main causes in both datasets.

CONCLUSIONS: This multinational study brings to focus the relation between allergic diseases and IEI. Major allergies do occur in IEI patients but were less frequent than the general population. Initial presentation with allergy could adversely affect the timely diagnosis of IEI. There is a need for policies to raise awareness and educate primary care and other referring specialties on the association of allergic diseases with IEI. This study provides a network among centers for future prospective studies in the field.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.