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An expanded guideline providing evidence-based recommendations for the management of adult and juvenile-onset Sjögren disease has been published by the British Society for Rheumatology (BSR).
Updated from the original 2017 version which focused on the systemic effects of the condition, the new guideline provides a framework for healthcare professionals to effectively and proactively manage individuals with Sjögren disease.
Its aim is to standardise care across the UK and enable non-specialist rheumatologists and allied healthcare professionals involved in the treatment of Sjögrens disease to manage patients holistically and in a personalised way.
The guideline working group established a set of 19 key questions around Sjögren disease diagnostics, comorbidities, clinically effective and timely treatments, considerations in pregnancy, non-pharmacological recommendations and tailored long-term follow-up.
They then interrogated the literature to determine the answers, which were then used to develop the recommendations.
For the first time, the guidelines include advice on Sjögren disease in adolescence and recurrent parotitis. They also note medication advancements such as access to ciclosporin for very severe eye dryness from four years of age and evidence around conventional and biologic disease-modifying antirheumatic drugs.
Dr Coziana Ciurtin, consultant in adult and adolescent rheumatology, said: ‘This is a first at an international level – there is no other guideline that looks at Sjögren disease across all ages. We recognise the need to incorporate recommendations for an under-diagnosed disease phenotype that starts in younger patients, who present slightly differently and may have different needs. We aim to support all clinicians and allied health professionals in diagnosing and looking after these younger patients.’
Changing the Sjögren disease emphasis
The guideline working group included members from rheumatology, including adult, adolescent and paediatric specialists, alongside a diverse working group consisting of GP, occupational therapy, ophthalmology, renal specialists and experts by experience.
As part of their work, the group took the decision to change the name of the condition from Sjögren syndrome to Sjögren disease. Dr Elizabeth Price, consultant rheumatologist at Great Western Hospital who led the BSR guideline development, said: ‘We had feedback from [European colleagues] and the patients that they preferred the name disease as it changed the emphasis of the condition and there is a move away from eponymous syndromes.’
She added: ‘I think in time we might lose the disease as well and it might just become Sjögren but we felt it was a little bit too early to do that.’
Alongside the full guideline, the working group have produced an easy-access summary sheet, which is free to download, as well as an audit tool that supports clinicians in making a correct diagnosis.
The BSR has recently extended the data capture for the New Early Inflammatory Arthritis Audit to include patients with newly diagnosed connective tissue disorders, including Sjögren’s.
Speaking at a roundtable on the guideline’s publication, Dr Price said: ‘A plea from me: if you can put all your newly diagnosed Sjögren’s patients into the audit, that will help us tremendously. Can I urge you to use the most modern criteria, which are referred to in the guideline as the ACR-EULAR combined 2016 criteria. If you look at the audit tool that goes along with the guideline, the first item in the audit tool is have you met the guidelines and there’s a little tick box for you to do that.’
