Quality of life of X-linked agammaglobulinemia patients in the United Kingdom
Quality of life of X-linked agammaglobulinemia patients in the United Kingdom
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.
Shillitoe, Ben
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Bourne, Helen
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Stroud, Catherine
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Flood, Terry
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Buckland, Matthew
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Ip, Winnie
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Worth, Austen
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Hackett, Scott
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Herwadkar, Archana
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Coulter, Tanya
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Jolles, Stephen
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Garcez, Tomaz
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Moya, Eduardo
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Faust, Saul N.
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Pearce, Mark S.
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Gennery, Andrew R.
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4 May 2026
Shillitoe, Ben
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Bourne, Helen
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Stroud, Catherine
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Flood, Terry
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Buckland, Matthew
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Ip, Winnie
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Worth, Austen
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Hackett, Scott
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Herwadkar, Archana
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Coulter, Tanya
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Jolles, Stephen
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Garcez, Tomaz
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Moya, Eduardo
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Faust, Saul N.
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Pearce, Mark S.
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Gennery, Andrew R.
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Shillitoe, Ben, Bourne, Helen, Stroud, Catherine, Flood, Terry, Buckland, Matthew, Ip, Winnie, Worth, Austen, Hackett, Scott, Herwadkar, Archana, Coulter, Tanya, Jolles, Stephen, Garcez, Tomaz, Moya, Eduardo, Faust, Saul N., Pearce, Mark S. and Gennery, Andrew R.
(2026)
Quality of life of X-linked agammaglobulinemia patients in the United Kingdom.
Journal of Human Immunity, 2 (3), [e20250198].
(doi:10.70962/jhi.20250198).
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.
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jhi_20250198
- Version of Record
Text
UK XLA QoL_shillitoe_jhi_final
More information
Accepted/In Press date: 6 February 2026
e-pub ahead of print date: 19 February 2026
Published date: 4 May 2026
Identifiers
Local EPrints ID: 510244
URI: http://eprints.soton.ac.uk/id/eprint/510244
PURE UUID: 02d3daef-5388-44dd-9342-65ccc283582f
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Date deposited: 24 Mar 2026 17:36
Last modified: 25 Mar 2026 02:41
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Contributors
Author:
Ben Shillitoe
Author:
Helen Bourne
Author:
Catherine Stroud
Author:
Terry Flood
Author:
Matthew Buckland
Author:
Winnie Ip
Author:
Austen Worth
Author:
Scott Hackett
Author:
Archana Herwadkar
Author:
Tanya Coulter
Author:
Stephen Jolles
Author:
Tomaz Garcez
Author:
Eduardo Moya
Author:
Mark S. Pearce
Author:
Andrew R. Gennery
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