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A world-first guideline looking at Sjögren disease across all ages has been published by the British Society for Rheumatology to raise awareness of the condition among a wide range of healthcare professionals and ensure timely treatment for patients. Dr Elizabeth Price, the consultant rheumatologist who led the development, discusses the context around the guideline, the unmet need it aims to tackle and the expected impact on patients’ quality of life.
Sjögren disease (SD) is a chronic, autoimmune disease of unknown aetiology with a significant impact on quality of life and no curative treatment. Although sicca of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations are common.
Impact of Sjögren disease on the patient
SD significantly impacts patients’ quality of life. A literature review found that health-related quality of life (HRQoL) was markedly reduced in SD in multiple studies across many countries compared with healthy controls.1
The reduction in HRQoL was similar to that observed in other chronic diseases, such as rheumatoid arthritis and systemic lupus erythematosus, suggesting that it is not a ‘benign’ disease. This reduction in quality of life has been noted in multiple domains and across all populations studied worldwide.
Anxiety, depression, pain and fatigue are all increased in SD compared with healthy controls and significantly impact quality of life.2 The loss of taste and smell that accompanies SD also has a negative effect on quality of life,3 as does the ocular dryness.4,5
There is a significant reduction in sexual quality of life6 (11) due to the combined effects of vaginal dryness,7 atrophy8 and psychosocial factors such as coping strategies and illness perceptions.9
Systemic involvement, including nervous system manifestations such as peripheral neuropathy,10 respiratory system involvement11 and arthralgia,12 also negatively impacts quality of life.
A meta-analysis suggests an increase in cardiovascular13 and respiratory morbidity14 and a small excess mortality has been observed in people with SD,15 particularly in males and those with underlying lung disease.16
Systemic (extra-glandular) features affect at least 70% of patients and include inflammatory arthritis, skin involvement, haematological abnormalities, neuropathies, interstitial lung disease and B-cell lymphoma (5–10% lifetime risk).17,18
SD remains a chronic illness with no disease-modifying or curative treatments available to date. People can accumulate morbidity over time.
What are the unmet needs?
SD is an under-recognised condition with significant unmet needs. The underlying pathogenesis is the subject of ongoing research but, as with other autoimmune diseases, there are multiple factors involved, including genetic vulnerability, hormonal status and environmental insults to the immune system.
Research into pathogenetic mechanisms is ongoing, and studies investigating non-pharmacological treatments, novel biologic drugs and the repurposing of existing conventional and biologic immunosuppressive agents are underway.
The development of effective treatments has been hampered by the disease’s heterogeneity. We now understand that accurate stratification of patients into disease subgroups and collaborative studies are essential in providing large enough cohorts to demonstrate meaningful effects of interventions.
There is a need to develop better measures of disease activity as the currently used parameters do not include fatigue and dryness, underestimate the disease burden and are not sensitive to change.
The HQIP-mandated New Early Inflammatory Arthritis Audit covering England and Wales (NEIAA) has recently expanded to include new connective tissue disease diagnoses, including SD. Teams should be encouraged to record all new cases to build an accurate picture of the incidence of new presentations and current delays to diagnosis.
Rationale for developing the new guideline
Individuals with SD currently experience long delays in diagnosis and there is poor awareness of the condition and its management among healthcare professionals who often underestimate the burden of SD.
Patients struggle with the chronic nature of the condition and are frustrated by the paucity of treatments and lack of knowledge in the medical field.
Successful management requires the personalisation of care. Although sicca of the eyes and mouth are the classically described features, dryness of other mucosal surfaces and systemic manifestations, including fatigue and arthralgia, are common.
Therefore, the key management aims should be to empower individuals to manage their condition; conserving, replacing and stimulating secretions; preventing damage; and suppressing underlying systemic disease activity.
A new guideline from the British Society for Rheumatology aims to provide comprehensive advice on the diagnosis and management of SD in adults and juvenile-onset SD for rheumatology teams in the UK19 and expands on the recommendations of the original 2017 guideline on the management of adults.20
We know that with appropriate care, quality of life and long-term outcomes improve, and we hope that these guidelines will provide a framework for healthcare professionals to diagnose and manage those with SD effectively and proactively.
The full guideline provides detailed evidence-based advice, which is broken down into 19 key areas. The recommendations are summarised in the executive summary and are accompanied by a one-page summary highlighting the key steps in managing and treating patients with SD effectively.
Raising awareness of Sjögren disease
The British Society for Rheumatology guideline needs to be publicised and widely disseminated to ensure it reaches its target audience. This should include clinicians caring for individuals with SD and those not satisfying criteria but who present with sicca symptoms.
The audience will also include, but is not limited to, paediatric and adult rheumatologists, general practitioners, ophthalmologists, oral medicine specialists, dentists, opticians, optometrists, specialist nurses, allied healthcare professionals and patients themselves.
Raising awareness of SD and its management should promote earlier diagnosis and lead to better outcomes for these individuals.
Author
Elizabeth Price MBBCh PhD
Department of Rheumatology, Great Western Hospital NHS Foundation Trust, Swindon, UK
References
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17. Ramos-Casals M et al. Primary Sjogren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine.2008;87(4):210–9.
18. Ramos-Casals M et al. Systemic involvement in primary Sjogren’s syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology 2014;53(2):321–31.
19. Price E et al. British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren’s disease. Rheumatology 2024;00:1–31.
20. Price EJ et al. The British Society for Rheumatology guideline for the management of adults with primary Sjögren’s Syndrome. Rheumatology 2017;56(10):1828.
