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

British Society for Rheumatology (BSR) · 2024 · United Kingdom

Source and licence

Authors
Elizabeth J Price, Stuart Benjamin, Michele Bombardieri et al.
Title
British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease
Journal
Rheumatology (Oxford, England), 2024
Licence
CC BY 4.0
Changes
Verbatim excerpts; formatting adapted

Full text retrieved 2026-10-27 from Europe PMC · PMC12013823

Licence statement of the original publication

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited.

This page reproduces excerpts of the original publication; the original is authoritative. It has not been reviewed by a physician, does not state a recommendation of Medelix and does not replace medical advice. The guideline may have been updated since.

Open the original publication →Full text on Europe PMC

Recommendations

Verbatim, with grade of recommendation or strength of consensus where given.

Recommendation

  • We recommend that the following additional investigations are undertaken at baseline, and repeated as clinically indicated, to detect comorbidities and associated autoimmune diseases: • Vitamin D levels; (1, B) (SOA 95.6%) • Thyroid function • Liver function tests (and anti-mitochondrial antibodies if indicated) • TTG • Immunoglobulins and serum electrophoresis • Serum bicarbonate • Creatine Kinase

Punctal occlusion

  • Expert opinion is that punctal plugs are suitable in certain circumstances but they may make corneal surface inflammation worse in certain situations. Careful patient selection is important.

Recommendation

  • We recommend an individualized holistic review for those with fatigue focusing on activity management (for example planning, prioritizing, pacing), sleep quality and lifestyle (2, C)SOA 96.7%

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.

Contents of the original publication

The section headings of the original.

  • Background and rationale for guideline development
  • Target audience
  • Guideline development, search methodology and dates
  • Key questions identified in the scope
  • 1. In people suspected of having SD, what is the diagnostic accuracy of ANA, ENA and other novel antigen testing?
  • Recommendation
  • 2a. In people suspected of having SD, what is the diagnostic accuracy of salivary gland US scanning?
  • Recommendation
  • 2b. In people suspected of having SD, what is the diagnostic accuracy of other imaging modalities?
  • Recommendation
  • 3a. In people suspected of having SD, what is the diagnostic accuracy of major and minor salivary gland biopsy?
  • Recommendation
  • 3b. In people suspected of having SD, what is the diagnostic accuracy of lacrimal gland biopsy?
  • Recommendation
  • 4a. In people with confirmed SD are there any measurable biomarkers that can predict development of lymphoma?
  • Recommendation
  • 4b. In people with confirmed SD are there any measurable biomarkers that can predict disease progression or development of extraglandular disease?
  • Recommendation
  • 5. In people with confirmed SD what other investigations should routinely be undertaken to exclude common associated conditions, for example coeliac or thyroid disease?
  • Recommendation
  • 6. In people with SD who have sicca (dryness) symptoms of the eyes, what is the most clinically effective topical treatment?
  • Recommendation
  • Recommendation
  • Recommendation
  • Recommendation
  • 7. In people with SD who have sicca (dryness) symptoms of the mouth, what is the most clinically effective topical treatment?
  • Recommendation
  • 8. In people with SD who have sicca (dryness) symptoms outside the eyes and mouth, what is the most clinically effective topical treatment?
  • 9a. In people with SD who have sicca (dryness) symptoms, what is the most clinically effective stimulatory treatment?
  • 9b. What is the clinical effectiveness of fluoride, xylitol, chlorhexidine, artificial saliva or diet in preventing the development or progression of dental caries and gum disease?
  • Recommendation
  • 10a. In people with SD what is the clinical effectiveness of treatments in comparison to each other or placebo for treating systemic disease?
  • 10b. What treatments are beneficial for recurrent parotitis in jSD?
  • Recommendation
  • 11. In people with SD, is early treatment of hypergammaglobulinaemia or systemic disease more effective than delayed treatment at slowing disease progression?
  • Recommendation
  • 12. What are the recommended therapeutic options in individuals with SD overlapping with other rheumatic diseases, for example, RA, SLE or scleroderma?
  • Recommendation
  • 13. In people with SD, what is the clinical effectiveness of nutraceuticals in the management of the condition?
  • Recommendation
  • 14. For people with SD, what cognitive therapy or behavioural change interventions are an effective treatment for fatigue and joint pain?
  • Recommendation
  • 15. In people with SD, what type and frequency of exercise is an effective treatment for fatigue?
  • Recommendation
  • 16. For pregnant people with SD, both with and without anti-Ro and/or La antibodies, is HCQ and/or low-dose aspirin effective in reducing fetal mortality and morbidity?
  • Recommendations
  • 17. For pregnant people with SD, with a fetus who has an incomplete heart block or hydropic changes, are fluorinated steroids and/or immunoglobulins effective in decreasing the likelihood of congenital heart block in the fetus?
  • Recommendation
  • 18. In people with SD, what is the most clinically effective long-term follow-up programme and how should this be personalized?
  • Recommendations
  • 19. What age-tailored information, education and support do people with SD and their families and carers need and how can they access this?
  • Recommendation
  • Applicability and utility
  • Research recommendations
  • Audit
  • Conclusions
  • Contributor Information

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