ArticlePloS one2022
Protocol for the unclassified primary antibody deficiency (unPAD) study: Characterization and classification of patients using the ESID online Registry.
Article in PloS one, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed.
- Low levels of IgG2 and pneumococcal antibodies as predictors of benefit from IgG replacement in IgG subclass deficiency.Journal of human immunity · 2026Article
- Low IgM Levels in Adult IEI: Classification Challenges and Clinical Implications.Journal of clinical immunology · 2026Article
- Exploring the Pathogens in Primary Predominantly Antibody Deficiencies of Unknown Genetic Origin.Journal of clinical immunology · 2025Article
- IPINeT Ped-unPAD Study: Goals, Design, and Preliminary Results.Journal of clinical medicine · 2024Article
- Cluster analysis of flowcytometric immunophenotyping with extended T cell subsets in suspected immunodeficiency.Immunity, inflammation and disease · 2023Article
- Selective IgM Deficiency: Evidence, Controversies, and Gaps.Diagnostics (Basel, Switzerland) · 2023Review
- The Evolutionary Scenario of Pediatric Unclassified Primary Antibody Deficiency to Adulthood.Journal of clinical medicine · 2023Article
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Authors and funding
7 authors.
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Abstract
backgroundPrimary antibody deficiencies (PADs) without an identified monogenetic origin form the largest and most heterogeneous group of primary immunodeficiencies. These patients often remain undiagnosed for years and many present to medical attention in adulthood after several infections risking structural complications. Not much is known about their treatment, comorbidities, or prognosis, nor whether the various immunological forms (decreased total IgG, IgG subclass(es), IgM, IgA, specific antibody responses, alone or in combination(s)) should be considered as separate, clearly definable subgroups. The unclassified primary antibody deficiency (unPAD) study aims to describe in detail all PAD patients without an identified specific monogenetic defect regarding their demographical, clinical, and immunological characteristics at presentation and during follow-up. In constructing these patterns, the unPAD study aims to reduce the number of missed and unidentified PAD patients in the future. In addition, this study will focus on subclassifying unPAD to support the identification of patients at higher risk for infection or immune dysregulation related complications, enabling the development of personalized follow-up and treatment plans. METHODS AND ANALYSIS: We present a protocol for a multicenter observational cohort study using the ESID online Registry. Patients of all ages who have given informed consent for participation in the ESID online Registry and fulfill the ESID Clinical Working Definitions for 'unclassified antibody deficiency', 'deficiency of specific IgG', 'IgA with IgG subclass deficiency', 'isolated IgG subclass deficiency', 'selective IgM deficiency', 'selective IgA deficiency' or 'common variable immunodeficiency' will be included. For all patients, basic characteristics can be registered at first registration and yearly thereafter in level 1 forms. Detailed characteristics of the patients can be registered in level 2 forms. Consecutive follow-up forms can be added indefinitely. To ensure the quality of the collected data, all data will be fully monitored before they are exported from the ESID online Registry for analysis. Outcomes will be the clinical and immunological characteristics of unPAD at presentation and during follow-up. Subgroup analyses will be made based on demographical, clinical and immunological characteristics.
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