Sjögren syndrome
Sicca plus systemic disease, the 2016 ACR/EULAR classification score, the extraglandular features examiners like (renal tubular acidosis, neuropathy, ILD, cryoglobulins), lymphoma red flags, anti-Ro in pregnancy, and what the drugs can and can't fix.
Tier 1Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- A chronic autoimmune disease of exocrine glands. Dry eyes and dry mouth in nearly all, systemic disease in about a third to a half, and a raised lymphoma risk.
- Women outnumber men about 9 to 1, peak onset 40 to 60. International consensus and the BSR 2025 guideline now call it Sjögren disease.
- Primary or associated with RA, SLE or systemic sclerosis.
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Sources
- Therapeutic Guidelines: Rheumatology (Sjögren syndrome)AU/NZTherapeutic Guidelines Limited (eTG complete), current online version, accessed November 2026
- Price EJ et al. British Society for Rheumatology guideline on management of adult and juvenile onset Sjögren disease. Rheumatology (Oxford) 2025;64:409-39British Society for Rheumatology, 2025
- Ramos-Casals M et al. EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies. Ann Rheum Dis 2020;79:3-18EULAR, 2019 (current EULAR version as of November 2026)
- Shiboski CH et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome. Ann Rheum Dis 2017;76:9-16ACR and EULAR, 2016
- Mariette X et al. Ianalumab for the treatment of patients with Sjögren's disease (NEPTUNUS-1 and NEPTUNUS-2). N Engl J Med 2026New England Journal of Medicine, 2026
- Gottenberg JE et al. Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome: the JOQUER randomized clinical trial. JAMA 2014;312:249-58JAMA, 2014
- Izmirly P et al. Hydroxychloroquine to prevent recurrent congenital heart block in fetuses of anti-SSA/Ro-positive mothers (PATCH). J Am Coll Cardiol 2020;76:292-302Journal of the American College of Cardiology, 2020
- Last updated
- 14 November 2026
What changed
- 14 November 2026 Personal emails, About story and Australian-style copy