by JoAnn Gurenlian, RDH, MS, PhD, AAFAAOM, FADHA
April 16, 2024

Sjögren disease (SD) is a chronic, autoimmune disease that impacts quality of life presenting with dryness (sicca) of the eyes and mouth, dryness of the mucosal surfaces and a variety of systemic manifestations including fatigue, arthralgia, arthritis, skin involvement, lung disease, and a lifetime risk of B-cell lymphoma. SD may occur alone or with rheumatic disease. Guidelines for management of SD were published in 2017 and the British Society of Rheumatology updated this information based on new evidence and using the AGREE II (Appraisal of guidelines for Research and Evaluation II) methodology.

A summary of the guideline for the management of adult and juvenile onset SD follows.

Step One

Confirm diagnosis:

  • Anti-Ro antibodies (score 3)
  • Focus score of > or = 1 (score 3)
  • Abnormal ocular staining score > or = 5 (score 1)
  • Schirmer’s test without anaesthetic result of < or = 5mm/5 min (score 1)
  • Unstimulated salivary flow ,0.1ml/min (score 1)

Classification as SD requires a score of 4 or more.

If no access to lip biopsy consider ultrasound as an alternative to support diagnosis.

Check for co-morbidities – clinical exam, urine dip, routine bloods, immunoglobulins, vitamin D, anti-dsDNA.

Explain diagnosis and signpost to appropriate resources.

Step Two

Treat symptoms

  • Dry eyes – start preservative-free lubricant eye drops 4 times per day, advise warm eye compress for 10 min daily
  • Dry mouth – saliva substitutes, dental care
  • Systemic dryness – consider pilocarpine 5 mg once daily increasing step-wise to 5 mg 3 times per day (max 5 mg x 6 daily)

Step Three

Systemic management:

  • Consider hydroxychloroquine
  • Consider other DMARDS for specific indications (see guideline)

Step Four

Extras and special situations:

  • If planning pregnancy – counsel re: neonatal lupus and congenital heart block
  • If co-morbidities – treat appropriately (see guideline)
  • Early diagnosis of lymphoma is critical for curative management. See guidelines for warning signs and symptoms

Lifestyle

  • Long-term monitoring of the condition is required, especially in those at high risk of lymphoma
  • Wear glasses to reduce tear evaporation
  • Maximise omega 3 through diet or supplements
  • Avoid dry, smoky environments
  • Humidify environment – turn down heating, saucers of water on radiators
  • Reduce sugar consumption
  • Meticulous dental care
  • Drink plain water
  • Keep active

To read the full guideline document, access it at rheumatology.org.uk/guidelines.

The BSR recognized that there are significant unmet needs in the management of patients with SD and further research is needed. There is a need to research pathogenic mechanisms tat may facilitate the development of targeted treatments. Better measures of disease activity are needed as the current parameters do not include fatigue and dryness. These measures underestimate the disease burden and are not sensitive to change. Further, there is a need to develop Quality Standards for SD which will improve standards of care.

The guidelines noted above are meant to provide a framework for health professionals to use to manage those with SD effectively and proactively. To learn more about the development of these guidelines and the recommendations of the BSR, access the article online.

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This article summarizes the “British Society for Rheumatology Guideline on Management of Adult and Juvenile Onset Sjögren Disease,” as published online in Rheumatology, April 16, 2024 (https://academic.oup.com/rheumatology/advance-article/doi/10.1093/rheumatology/keae152/7645909).

JoAnn Gurenlian, RDH, MS, PhD, AAFAAOM, FADHA, is the ADHA Director of Education, Research and Advocacy, professor emerita in the Department of Dental Hygiene at ISU, and former president of the American Dental Hygienists’ Association 1990-1991.