NDLI: Mild Hypogammaglobulinemia Can Be a Serious Condition

Content Provider	Directory of Open Access Journals (DOAJ)
Author	Lisanne M. A. Janssen Paul Bassett Thomas Macken Jolanda van Esch Hans Pruijt Arnoud Knoops Markus Sköld Antony Parker Jolanda de Vries Esther de Vries
Abstract	Background: Most patients with primary antibody deficiency (PAD) suffer from less well-described and understood forms of hypogammaglobulinemia (unclassified primary antibody deficiency, unPAD). Because of the moderately decreased immunoglobulin levels compared to CVID, unPAD is generally considered to be clinically mild and not very relevant.Objective: To describe our cohort of—mainly—unPAD patients, and to analyze whether subgroups can be identified.Methods: Data were prospectively collected (February-2012 to June-2016) as part of a standardized, 1-day Care Pathway for suspected primary immunodeficiency. The TNO-AZL Questionnaire for Health-Related Quality of Life (HRQoL) was part of the pre-first-visit intake procedure.Results: Three hundred and twenty patients were referred to the Care Pathway. Data from 23/27 children and 99/113 adults who were diagnosed with PAD and gave informed consent were available for analysis. 89/99 adults had unPAD, the majority (74%) were female and 44% already showed bronchiectasis. HRQoL was significantly decreased in all domains, meaning that a lot of unPAD patients had to cope simultaneously with pain, negative feelings and impairments in cognition, home management tasks, sleep, social interaction, and work. The most prominently impaired HRQoL domain was vitality, indicating these patients feel extremely tired and worn out.Conclusion: These results highlight the need for more attention to the potential patient burden of unPADs. A larger cohort is needed to increase our understanding of unPADs and to analyze whether distinct subgroups can be identified. For now, it is important for the clinician to acknowledge the existence of unPAD and be aware of its potential consequences, in order to timely and appropriately manage its effects and complications.
e-ISSN	16643224
DOI	10.3389/fimmu.2018.02384
Journal	Frontiers in Immunology
Volume Number	9
Language	English
Publisher	Frontiers Media S.A.
Publisher Date	2018-10-01
Publisher Place	Switzerland
Access Restriction	Open
Subject Keyword	Immunologic Diseases. Allergy Primary Antibody Deficiency Immunodeficiency Unclassified Primary Antibody Deficiency Primary Immunodeficiency Hypogammaglobulinemia Common Variable Immunodeficiency Disorders
Content Type	Text
Resource Type	Article

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