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A systematic review of primary Sjögren’s syndrome in male and paediatric populations

Virdee, S; Greenan-Barrett, J; Ciurtin, C; (2017) A systematic review of primary Sjögren’s syndrome in male and paediatric populations. Clinical Rheumatology , 36 (10) pp. 2225-2236. 10.1007/s10067-017-3745-z. Green open access

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Abstract

Primary Sjögren’s syndrome (pSS) is a chronic multisystem autoimmune rheumatic disease characterised by female predominance. Although the disease is rare in the male and paediatric populations, it has been suggested that it may have a different disease phenotype, which has not been investigated before using a systematic approach. A systematic literature search of PubMed databases (updated to December 2016) was performed to identify all published data on the epidemiological, clinical and laboratory manifestations of pSS in the male and paediatric populations. The literature search of the male and paediatric pSS studies identified 2025 and 186 citations, respectively, out of which 7 and 5 fulfilled our inclusion criteria and were analysed further. The range of age at disease onset was 9.4–10.7 years for children and 39.4–56.9 years at diagnosis for male patients. We identified a prevalence of extra-glandular manifestations between 52.6–92.3% in the male population and 50.0–84.6% in children, while abnormal sialometry was only reported in the paediatric population, with a prevalence between 71.4 and 81.8%. There was a significant variation of positive serological markers, with anti-Ro antibodies reported between 15.7–75.0% and 36.4–84.6%, and anti-La antibodies between 5.6–51.7% and 27.3–65.4%, in the male and paediatric populations, respectively. The characteristics of pSS in the male and paediatric populations varied according to different studies. When compared to data available from pSS adult populations, children diagnosed with pSS reported less dryness and had a higher prevalence of parotitis, lymphadenopathy and systemic symptoms and male patients were younger at the time of diagnosis. This systematic review contributes to a better understanding of the epidemiology of pSS in rare populations. Large longitudinal cohort studies comparing male with female patients and adult with paediatric patients are needed.

Type: Article
Title: A systematic review of primary Sjögren’s syndrome in male and paediatric populations
Open access status: An open access version is available from UCL Discovery
DOI: 10.1007/s10067-017-3745-z
Publisher version: http://doi.org/10.1007/s10067-017-3745-z
Language: English
Additional information: © 2017 The Author(s). This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
Keywords: Primary Sjögren’s syndrome; Male patients; Children; Systematic review
UCL classification: UCL
UCL > Provost and Vice Provost Offices
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/1561492
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