Table 1

Overarching (A–C) and specific (1–12) recommendations

LoEGoRVote (%)LoA (0–10)
A.Patients with SjS should be managed at, or in close collaboration with, centres of expertise following a multidisciplinary approachNANA909.2
B.The first therapeutic approach for dryness should be symptomatic relief using topical therapiesNANA938.9
C.Systemic therapies may be considered for the treatment of active systemic diseaseNANA909.1
1.Baseline evaluation of salivary gland function is recommended before starting treatment for oral dryness5D818.7
2.The preferred first therapeutic approach for oral dryness according to salivary gland function may be:1a/*1bB888.7
 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/ointments1aB989.5
4.Refractory/severe ocular dryness may be managed using topical immunosuppressive-containing drops* and autologous serum eye drops1a/*1bB/D949.1
5.Concomitant diseases should be evaluated in patients presenting with fatigue/pain, whose severity should be scored using specific tools5D939.0
6.Consider analgesics or other pain-modifying agents for musculoskeletal pain, considering the balance between potential benefits and side-effects4C898.9
7.Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions4C899.0
8.Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease4C859.6
9.Immunosuppressive agents should be mainly used as GC-sparing agents, with no evidence supporting the choice of one agent over another4C828.9
10.B-cell targeted therapies may be considered in patients with severe, refractory systemic disease1bB988.6
11.The systemic organ-specific therapeutic approach may follow, as a general rule, the sequential (or combined) use of GCs, immunosuppressive agents and biologics5D988.6
12.Treatment of B-cell lymphoma should be individualised according to the specific histological subtype and disease stage4C889.7
  • 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.