Overarching (A–C) and specific (1–12) recommendations
| LoE | GoR | Vote (%) | LoA (0–10) | |
| A.Patients with SjS should be managed at, or in close collaboration with, centres of expertise following a multidisciplinary approach | NA | NA | 90 | 9.2 |
| B.The first therapeutic approach for dryness should be symptomatic relief using topical therapies | NA | NA | 93 | 8.9 |
| C.Systemic therapies may be considered for the treatment of active systemic disease | NA | NA | 90 | 9.1 |
| 1.Baseline evaluation of salivary gland function is recommended before starting treatment for oral dryness | 5 | D | 81 | 8.7 |
| 2.The preferred first therapeutic approach for oral dryness according to salivary gland function may be: | 1a/*1b | B | 88 | 8.7 |
| 2.1. Non-pharmacological stimulation for mild dysfunction; | ||||
| 2.2. Pharmacological stimulation* for moderate dysfunction; | ||||
| 2.3. Saliva substitution for severe dysfunction | ||||
| 3.The first-line therapeutic approach to ocular dryness includes the use of artificial tears and ocular gels/ointments | 1a | B | 98 | 9.5 |
| 4.Refractory/severe ocular dryness may be managed using topical immunosuppressive-containing drops* and autologous serum eye drops | 1a/*1b | B/D | 94 | 9.1 |
| 5.Concomitant diseases should be evaluated in patients presenting with fatigue/pain, whose severity should be scored using specific tools | 5 | D | 93 | 9.0 |
| 6.Consider analgesics or other pain-modifying agents for musculoskeletal pain, considering the balance between potential benefits and side-effects | 4 | C | 89 | 8.9 |
| 7.Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions | 4 | C | 89 | 9.0 |
| 8.Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease | 4 | C | 85 | 9.6 |
| 9.Immunosuppressive agents should be mainly used as GC-sparing agents, with no evidence supporting the choice of one agent over another | 4 | C | 82 | 8.9 |
| 10.B-cell targeted therapies may be considered in patients with severe, refractory systemic disease | 1b | B | 98 | 8.6 |
| 11.The systemic organ-specific therapeutic approach may follow, as a general rule, the sequential (or combined) use of GCs, immunosuppressive agents and biologics | 5 | D | 98 | 8.6 |
| 12.Treatment of B-cell lymphoma should be individualised according to the specific histological subtype and disease stage | 4 | C | 88 | 9.7 |
LoE and GoR according to the Oxford Centre for Evidence-based Medicine—LoE (March 2009). Vote (%): % of participants scoring the recommendation as at least ‘important’ (score of ≥4 on 5-point scale). LoA: mean score (scale of ‘0’ as no agreement, ‘10’ full agreement).
ESSDAI, EULAR Sjögren's syndrome disease activity index; EULAR, European League Against Rheumatism; GC, glucocorticoid; GoR, grade of recommendation; LoA, levels of agreement; LoE, levels of evidence; NA, not applicable.