Quality of life of X-linked agammaglobulinemia patients in the United Kingdom
Shillitoe, Ben Bourne, Helen Stroud, Catherine Flood, Terry Buckland, Matthew Ip, Winnie Worth, Austen Hackett, Scott Herwadkar, Archana Coulter, Tanya
Shillitoe, Ben
Bourne, Helen
Stroud, Catherine
Flood, Terry
Buckland, Matthew
Ip, Winnie
Worth, Austen
Hackett, Scott
Herwadkar, Archana
Coulter, Tanya
Abstract
Due to limitations in current therapies (immunoglobulin replacement therapy), complications in X-linked agammaglobulinemia (XLA) such as bronchiectasis may continue to occur, with subsequent significant impacts on health-related quality of life (HRQoL). There were no significant differences in PedsQL 4.0 total scores against UK healthy norms (self 80.98, P = 0.277; parent 79.76, P = 0.465). There were no significant differences in the SF36v2 physical component score (PCS) against UK healthy norms (49.10, P = 0.712). However, XLA patients with bronchiectasis had significantly worse PCS than patients without (47.88 versus 55.14, P < 0.001) and significantly worse PCS than UK healthy norms (P = 0.004). In the absence of bronchiectasis, HRQoL is comparable to UK healthy norms. However, XLA patients with bronchiectasis have significantly worse HRQoL than patients without bronchiectasis and UK healthy norms. These data demonstrate that further work into novel therapies are needed to prevent bronchiectasis to enable XLA patients to have a normal quality of life.
MIDER Authors
Affiliations
Sheffield Children's NHS Foundation Trust; Newcastle Hospitals NHS Foundation Trust; University College London; Great Ormond Street Hospital for Children NHS Foundation Trust; University Hospitals Birmingham NHS Foundation Trust; Northern Care Alliance NHS Trust; Belfast Health and Social Care Trust; Cardiff and Vale University Health Board; Manchester University NHS Foundation Trust; Bradford Teaching Hospitals NHS Foundation Trust; University Hospital Southampton NHS Foundation Trust; University of Southampton; Newcastle University
Date
2026-02-19
Type
Article
Collections
Citation
Shillitoe B, Bourne H, Stroud C, Flood T, Buckland M, Ip W, Worth A, Hackett S, Herwadkar A, Coulter T, Jolles S, Garcez T, Moya E, Faust SN, Pearce MS, Gennery AR. Quality of life of X-linked agammaglobulinemia patients in the United Kingdom. J Hum Immun. 2026 Feb 19;2(3):e20250198. doi: 10.70962/jhi.20250198.