2019 EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies

Algorithm of glandular function assessment and therapeutic approach in patients with primary SjS presenting with oral dryness. SjS, Sjögren syndrome; SWSF, stimulated whole salivary flows; UWSF, unstimulated whole salivary flows.

Table adapted from: Ramos-Casals M, Brito-Zerón P, Bombardieri S On behalf of the EULAR-Sjögren Syndrome Task Force Group, et al. “EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies.” Annals of the Rheumatic Diseases 2020;79:3-18.

Algorithm of glandular function assessment and therapeutic approach in patients with primary SjS presenting with ocular dryness. *Consider neuropathic pain if OSS≤1. **Additional criteria for severity: (1) impaired visual function (photophobia, visual acuity modification or low contrast sensitivity); (2) blepharospasm (secondary to ocular inflammation); (3) severe meibomian gland disease or eyelid inflammation. ***For short-term indications (2–4 weeks). CyA, ciclosporin A; GC, glucocorticoid; OSS, ocular staining score (Whitcher107 JP, et al. Am J Ophthalmol. 2010;149:405–15). OSDI, ocular surface disease index (adapted from Baudouin C,108 et al. Br J Ophthalmol 2014;98:1168–1176); SjS, Sjögren syndrome.

Table adapted from: Ramos-Casals M, Brito-Zerón P, Bombardieri S On behalf of the EULAR-Sjögren Syndrome Task Force Group, et al. “EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies.” Annals of the Rheumatic Diseases 2020;79:3-18.

A to I) Algorithm for the therapeutic approach to patients with primary SjS presenting with organ-specific systemic involvements. NSAIDs: no longer than 7–10 days. HCQ: hydroxychloroquine 200 mg/day. GC (recommended dose in mg/kg/day); short-term course whenever possible; consider methylprednisolone pulses in severe cases. ID: immunosuppressive agents, no head-to-head comparisons. CyC: cyclophosphamide pulses 0.5 g/15 day (maximum six pulses). Rituximab: rituximab 1 g/15 days (x2). BLM: belimumab; 10 mg/kg (0, 2 and 4 weeks and then every 4 weeks). ABA: abatacept 0, 2, 4 weeks and every 4 weeks. IVIg: intravenous immunoglobulins 0.4–2 g/kg 5 days. PEX: plasma exchanges. CyA, ciclosporin A; EULAR, European League Against Rheumatism; ESSDAI, EULAR Sjögren”s syndrome disease activity index; GC, glucocorticoid; LoE, levels of evidence; NSAIDs, non-steroidal anti-inflammatory drugs; RA, rheumatoid arthritis; SjS, Sjögren syndrome.

Table adapted from: Ramos-Casals M, Brito-Zerón P, Bombardieri S On behalf of the EULAR-Sjögren Syndrome Task Force Group, et al. “EULAR recommendations for the management of Sjögren’s syndrome with topical and systemic therapies.” Annals of the Rheumatic Diseases 2020;79:3-18.