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Barriers to translational research in Sjӧgren’s syndrome with childhood onset; challenges of recognising and diagnosing an orphan rheumatic disease

Ciurtin, C; Cho, Y; Al-Obaidi, M; Jury, E; Price, E; (2021) Barriers to translational research in Sjӧgren’s syndrome with childhood onset; challenges of recognising and diagnosing an orphan rheumatic disease. Lancet Rheumatology , 3 (2) e138-e148. 10.1016/S2665-9913(20)30393-3. Green open access

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Abstract

Sjögren's syndrome was considered for many years a disease of adulthood, characterised by immune infiltration of exocrine glands, leading to dryness (eg, dry mouth and eyes), which is a cardinal symptom. As of the last 20 years, it became apparent that although the disease is very rare in children, its clinical presentation differs from that of adults, posing substantial challenges to the recognition, diagnosis, and classification of patients with childhood-onset Sjögren's syndrome. This Viewpoint explores comparative classification criteria for children (not validated) and adults with Sjögren's syndrome, as well as differences in the clinical presentation of childhood-onset versus adult-onset Sjögren's syndrome, offering ideas about how we can improve the diagnosis of Sjögren's syndrome in children. A review of the role of medical history and clinical assessment, serology, glandular function assessment, and imaging, as well as salivary and lachrymal gland biopsy in the diagnosis of children with Sjögren's syndrome is included. Additionally, we provide suggestions about further research and registry data collection that is required to address the unmet needs of these patients.

Type: Article
Title: Barriers to translational research in Sjӧgren’s syndrome with childhood onset; challenges of recognising and diagnosing an orphan rheumatic disease
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/S2665-9913(20)30393-3
Publisher version: https://doi.org/10.1016/S2665-9913(20)30393-3
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10117792
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