Sjögren syndrome
Also known as: sicca syndrome · Sjogren
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Treatment Guidelines for Rheumatologic Manifestations of Sjögren's Syndrome: Use of Biologic Agents, Management of Fatigue, and Inflammatory Musculoskeletal Pain
Sjögren's Foundation · 2017
The Sjögren's Foundation's recommendations for the systemic, rheumatology-facing side of Sjögren's disease. Developed by a panel of clinicians, nurses and patients using a modified Delphi process, it produced nineteen recommendations covering biologic therapy, persistent fatigue and inflammatory joint and muscle pain. It is the US counterpart to the systemic-therapy half of EULAR's recommendations, though it does not cover dry eye and dry mouth.
- Hydroxychloroquine is positioned as the first drug to try for inflammatory musculoskeletal pain in primary Sjögren's. source
- Hydroxychloroquine is also suggested for fatigue, on the strength of clinical experience and its safety record rather than strong trial evidence. source
- Rituximab carries a moderately strong recommendation for severe systemic disease — vasculitis, cryoglobulinaemia, major salivary gland swelling, inflammatory arthritis, lung involvement or peripheral neuropathy — based largely on non-randomised data. source
- Nineteen recommendations were agreed by a mixed clinician, nurse and patient panel using a modified Delphi process with a 75% agreement threshold. source
EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies
European Alliance of Associations for Rheumatology (EULAR) · 2020 · 2019 recommendations (published 2020)
The first EULAR evidence- and consensus-based recommendations on drug treatment of Sjögren's syndrome, published in Annals of the Rheumatic Diseases. They move from management of the core symptoms of dryness, fatigue and pain through to treatment of systemic disease manifestations.
- Structured as three overarching recommendations plus 12 specific recommendations in a stepwise sequence. source
- Covers topical ocular and oral therapies, muscarinic agonists, hydroxychloroquine, glucocorticoids, synthetic immunosuppressants and biologic agents. source
- Levels of evidence were generally modest while task-force agreement was generally very high. source
US vs EU key differences
- The two documents cover different amounts of ground: EULAR spans the whole disease, with topical management of dry eye and dry mouth (artificial tears, topical ciclosporin and corticosteroids, saliva substitutes, oral muscarinic agonists) alongside systemic therapy, while the US Sjögren's Foundation guideline addressed here is limited to rheumatologic and systemic manifestations and issues no topical sicca recommendations. source
- Rituximab is positioned far more permissively in the US document, which lists it for selected patients with oral and ocular dryness and for extraglandular disease including vasculitis, severe parotid swelling, inflammatory arthritis, pulmonary involvement and mononeuritis multiplex. EULAR confines B-cell-targeted therapy to patients with severe, refractory systemic disease. source
- EULAR's task force restricts rituximab to severe, refractory systemic disease rather than offering it as a treatment for sicca symptoms, a narrower indication than the US Foundation guideline's dryness recommendation. source
- Only the US guideline takes an explicit negative position on TNF inhibitors, strongly discouraging their use for sicca symptoms and in most clinical contexts in primary Sjögren's syndrome. source
- Fatigue is handled at different levels: the US guideline dedicates recommendations to non-pharmacologic management, advising self-care measures and exercise, while EULAR folds fatigue into a sequenced approach to the central symptom triplet of dryness, fatigue and pain before systemic disease is addressed. source
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