British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease

doi:10.1093/rheumatology/keae152

Practice Guideline

. 2025 Feb 1;64(2):409-439.

doi: 10.1093/rheumatology/keae152.

British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease

Elizabeth J Price¹, Stuart Benjamin², Michele Bombardieri^{3

4}, Simon Bowman^{5

6

7}, Sara Carty¹, Coziana Ciurtin⁸, Bridget Crampton⁹, Annabel Dawson¹⁰, Benjamin A Fisher^{11

12}, Ian Giles⁸, Peter Glennon¹³, Monica Gupta¹⁴, Katie L Hackett¹⁵, Genevieve Larkin¹⁶, Wan-Fai Ng^{17

18}, Athimalaipet V Ramanan^{19

20}, Saad Rassam²¹, Saaeha Rauz^{22

23}, Guy Smith²⁴, Nurhan Sutcliffe²⁵, Anwar Tappuni²⁶, Stephen B Walsh²⁷

Affiliations

¹ Department of Rheumatology, Great Western Hospital NHS Foundation Trust, Swindon, UK.
² The Academy Library and Information Service, Great Western Hospital NHS Foundation Trust, Swindon, UK.
³ Department of Rheumatology, Barts and The London School of Medicine and Dentistry, Barts Health NHS Trust, London, UK.
⁴ Centre for Experimental Medicine and Rheumatology, The William Harvey Research Institute, Queen Mary University of London, London, UK.
⁵ Department of Rheumatology, Milton Keynes University Hospital, Milton Keynes, UK.
⁶ Department of Rheumatology, University Hospitals Birmingham NHSFT, Birmingham, UK.
⁷ Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
⁸ Centre for Rheumatology, Division of Medicine, University College London, London, UK.
⁹ Patient Representative, Sjogren's UK Helpline Lead, Sjogren's UK (British Sjögren's Syndrome Association), Birmingham, UK.
¹⁰ Patient Representative, Sjogren's UK (British Sjögren's Syndrome Association), Birmingham, UK.
¹¹ Rheumatology Research Group, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
¹² National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre and Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
¹³ General Practice, NHS Staffordshire & Stoke on Trent ICB, Stafford, UK.
¹⁴ Department of Rheumatology, Gartnavel General Hospital, Glasgow, UK.
¹⁵ Department of Social Work, Education and Community Wellbeing, Northumbria University, Newcastle upon Tyne, UK.
¹⁶ Department of Ophthalmology, Kings College Hospital, London, UK.
¹⁷ Translational and Clinical Research Institute & Newcastle NIHR Biomedical Research Centre, Newcastle University, Newcastle upon Tyne, UK.
¹⁸ Department of Rheumatology, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, UK.
¹⁹ Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK.
²⁰ Translational Health Sciences, University of Bristol, Bristol, UK.
²¹ Haematology and Haemato-Oncology, KIMS Hospital, Maidstone, Kent, UK.
²² Ophthalmology, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
²³ Birmingham and Midland Eye Centre, Sandwell and West Birmingham NHS Trust, Birmingham, UK.
²⁴ Department of Ophthalmology, Great Western Hospital NHS Foundation Trust, Swindon, UK.
²⁵ Department of Rheumatology, Barts Health NHS Trust, London, UK.
²⁶ Institute of Dentistry, Queen Mary University of London, London, UK.
²⁷ London Tubular Centre, University College London, London, UK.

PMID: 38621708
PMCID: PMC12013823
DOI: 10.1093/rheumatology/keae152

Practice Guideline

British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease

Elizabeth J Price et al. Rheumatology (Oxford). 2025.

. 2025 Feb 1;64(2):409-439.

doi: 10.1093/rheumatology/keae152.

Authors

Affiliations

¹ Department of Rheumatology, Great Western Hospital NHS Foundation Trust, Swindon, UK.
² The Academy Library and Information Service, Great Western Hospital NHS Foundation Trust, Swindon, UK.
³ Department of Rheumatology, Barts and The London School of Medicine and Dentistry, Barts Health NHS Trust, London, UK.
⁴ Centre for Experimental Medicine and Rheumatology, The William Harvey Research Institute, Queen Mary University of London, London, UK.
⁵ Department of Rheumatology, Milton Keynes University Hospital, Milton Keynes, UK.
⁶ Department of Rheumatology, University Hospitals Birmingham NHSFT, Birmingham, UK.
⁷ Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
⁸ Centre for Rheumatology, Division of Medicine, University College London, London, UK.
⁹ Patient Representative, Sjogren's UK Helpline Lead, Sjogren's UK (British Sjögren's Syndrome Association), Birmingham, UK.
¹⁰ Patient Representative, Sjogren's UK (British Sjögren's Syndrome Association), Birmingham, UK.
¹¹ Rheumatology Research Group, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
¹² National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre and Department of Rheumatology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
¹³ General Practice, NHS Staffordshire & Stoke on Trent ICB, Stafford, UK.
¹⁴ Department of Rheumatology, Gartnavel General Hospital, Glasgow, UK.
¹⁵ Department of Social Work, Education and Community Wellbeing, Northumbria University, Newcastle upon Tyne, UK.
¹⁶ Department of Ophthalmology, Kings College Hospital, London, UK.
¹⁷ Translational and Clinical Research Institute & Newcastle NIHR Biomedical Research Centre, Newcastle University, Newcastle upon Tyne, UK.
¹⁸ Department of Rheumatology, Newcastle upon Tyne NHS Foundation Trust, Newcastle upon Tyne, UK.
¹⁹ Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK.
²⁰ Translational Health Sciences, University of Bristol, Bristol, UK.
²¹ Haematology and Haemato-Oncology, KIMS Hospital, Maidstone, Kent, UK.
²² Ophthalmology, Institute of Inflammation and Ageing, University of Birmingham, Birmingham, UK.
²³ Birmingham and Midland Eye Centre, Sandwell and West Birmingham NHS Trust, Birmingham, UK.
²⁴ Department of Ophthalmology, Great Western Hospital NHS Foundation Trust, Swindon, UK.
²⁵ Department of Rheumatology, Barts Health NHS Trust, London, UK.
²⁶ Institute of Dentistry, Queen Mary University of London, London, UK.
²⁷ London Tubular Centre, University College London, London, UK.

PMID: 38621708
PMCID: PMC12013823
DOI: 10.1093/rheumatology/keae152

Abstract

Sjögren disease (SD) is a chronic, autoimmune disease of unknown aetiology with significant impact on quality of life. Although dryness (sicca) of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations are common. The key management aim should be to empower the individual to manage their condition-conserving, replacing and stimulating secretions; and preventing damage and suppressing systemic disease activity. This guideline builds on and widens the recommendations developed for the first guideline published in 2017. We have included advice on the management of children and adolescents where appropriate to provide a comprehensive guideline for UK-based rheumatology teams.

Keywords: Sjögren disease; Sjögren’s syndrome; connective tissue disease; guideline; management; recommendations; treatment.

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References

1. Price EJ, Rauz S, Tappuni AR et al.; British Society for Rheumatology Standards, Guideline and Audit Working Group. The British Society for Rheumatology guideline for the management of adults with primary Sjögren's Syndrome. Rheumatology (Oxford, England) 2017;56:1828. - PubMed
1. Price E, Allen A, Rauz S et al. The management of Sjögren’s syndrome: British Society for Rheumatology guideline scope. Rheumatology 2020;60:2122–7. - PubMed
1. Hackett K, Deary V, Deane K et al. SAT0736-HPR “like a bag of liquorice allsorts—everybody's got different flavours”: a qualitative focus group study to explore symptoms of fatigue, sleep disturbances and pain in primary Sjögren's syndrome patients and to develop a future model of care. Ann Rheum Dis 2017;76(Suppl 2):1517.
1. Ramos-Casals M, Solans R, Rosas J et al.; GEMESS Study Group. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine 2008;87:210–9. - PubMed
1. Ramos-Casals M, Brito-Zeron P, Solans R et al.; Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI). Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford, England) 2014;53:321–31. - PubMed

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[1] Price EJ, Rauz S, Tappuni AR et al.; British Society for Rheumatology Standards, Guideline and Audit Working Group. The British Society for Rheumatology guideline for the management of adults with primary Sjögren's Syndrome. Rheumatology (Oxford, England) 2017;56:1828. - PubMed

[2] Price EJ, Rauz S, Tappuni AR et al.; British Society for Rheumatology Standards, Guideline and Audit Working Group. The British Society for Rheumatology guideline for the management of adults with primary Sjögren's Syndrome. Rheumatology (Oxford, England) 2017;56:1828. - PubMed

[3] Price E, Allen A, Rauz S et al. The management of Sjögren’s syndrome: British Society for Rheumatology guideline scope. Rheumatology 2020;60:2122–7. - PubMed

[4] Price E, Allen A, Rauz S et al. The management of Sjögren’s syndrome: British Society for Rheumatology guideline scope. Rheumatology 2020;60:2122–7. - PubMed

[5] Hackett K, Deary V, Deane K et al. SAT0736-HPR “like a bag of liquorice allsorts—everybody's got different flavours”: a qualitative focus group study to explore symptoms of fatigue, sleep disturbances and pain in primary Sjögren's syndrome patients and to develop a future model of care. Ann Rheum Dis 2017;76(Suppl 2):1517.

[6] Hackett K, Deary V, Deane K et al. SAT0736-HPR “like a bag of liquorice allsorts—everybody's got different flavours”: a qualitative focus group study to explore symptoms of fatigue, sleep disturbances and pain in primary Sjögren's syndrome patients and to develop a future model of care. Ann Rheum Dis 2017;76(Suppl 2):1517.

[7] Ramos-Casals M, Solans R, Rosas J et al.; GEMESS Study Group. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine 2008;87:210–9. - PubMed

[8] Ramos-Casals M, Solans R, Rosas J et al.; GEMESS Study Group. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine 2008;87:210–9. - PubMed

[9] Ramos-Casals M, Brito-Zeron P, Solans R et al.; Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI). Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford, England) 2014;53:321–31. - PubMed

[10] Ramos-Casals M, Brito-Zeron P, Solans R et al.; Autoimmune Diseases Study Group (GEAS) of the Spanish Society of Internal Medicine (SEMI). Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford, England) 2014;53:321–31. - PubMed

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British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease

Affiliations

British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease

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