Table 1 European Alliance of Associations for Rheumatology (EULAR) recommendations for the management of primary Sjögren’s disease [67]

Oral and ocular dryness punctum plugs

• The first therapeutic approach for oral dryness according to salivary gland function may be:

– Non-pharmacological stimulation for mild dysfunction

– Pharmacological stimulation with muscarinic agonists (e.g., pilocarpine, cevimeline) for moderate dysfunction

– Saliva substitution for severe dysfunction

• The first-line therapeutic approach to ocular dryness includes the use of artificial tears and ocular gels or ointments. Refractory or severe ocular dryness may be managed using topical immunosuppressive-containing drops and autologous serum eye drops

Fatigue and pain

• The severity of fatigue and pain should be scored using specific tools. Concomitant diseases should be evaluated

• Analgesics or other pain-modifying agents should be considered for musculoskeletal pain

Systemic disease manifestations

• Treatment of systemic disease should be tailored to organ-specific severity using the EULAR Sjögren’s Syndrome Disease Activity Index definitions

• Glucocorticoids should be used at the minimum dose and length of time necessary to control systemic disease

• Immunosuppressive agents should mainly be used as glucocorticoid-sparing agents

• B cell-targeted therapies may be considered in patients with severe refractory systemic disease

• The organ-specific therapeutic approach may follow, as a general rule, the sequential (or combined) use of glucocorticoids, immunosuppressive agents, and biologic agents

B cell lymphoma

• Treatment of B cell lymphoma should be individualized according to histological subtype and disease stage