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sjogrens-syndrome
Sjögren's Syndrome
Primary Sjögren's syndrome (pSS): systemic lymphocytic exocrinopathy; xerostomia, xerophthalmia, anti-Ro/SSA (80%); type I IFN signature; 40× elevated lymphoma risk; BAFF drives B-cell hyperactivation. Ianalumab (anti-BAFFR; TWINSS 2023) and rituximab are active biologics.
Entry Metadata
| Field | Value |
|---|---|
| ID | sjogrens-syndrome |
| Name | Sjögren's Syndrome |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome. Arthritis Rheumatol. 2017;69(1):35-45. · PubMed 27785888
- Dörner T, Bowman SJ, Fox R, et al. Ianalumab (VAY736) in patients with primary Sjögren's syndrome: a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial (TWINSS). Lancet. 2023;402(10400):477-489. · PubMed 37499657
- Seror R, Ravaud P, Mariette X, et al. EULAR Sjögren's Syndrome Disease Activity Index and Patient Reported Index. Ann Rheum Dis. 2019;78(11):1554-1560. · PubMed 31462415