Treatment of Sjögren's Syndrome Flare

General Approach to Management

The European League Against Rheumatism recommends a multidisciplinary approach involving specialists in autoimmune diseases as coordinators, with shared decision-making between the patient and healthcare team 4
Treatment should be tailored to the specific manifestations present during the flare, with different approaches for sicca symptoms versus systemic disease 4

For mild salivary gland dysfunction, the American College of Rheumatology suggests non-pharmacological stimulation (sugar-free gum, frequent sips of water) 4
For moderate dysfunction, the European League Against Rheumatism recommends pharmacological stimulation with muscarinic agonists (pilocarpine or cevimeline) 4
For severe dysfunction, saliva substitutes are recommended 4
First-line treatment for ocular dryness includes artificial tears and ocular gels/ointments 4
For refractory or severe cases of ocular dryness, topical immunosuppressive-containing drops and autologous serum eye drops may be considered 4

The American College of Rheumatology recommends evaluating for concomitant conditions that may contribute to fatigue or pain 4
Score severity using specific tools, such as fatigue or pain scales 4
For musculoskeletal pain, consider analgesics or other pain-modifying agents, weighing benefits against side effects 4

Treatment should be tailored to organ-specific severity using ESSDAI definitions 4
Follow a sequential approach based on severity:
Glucocorticoids should be used at the minimum dose and for the shortest time necessary to control active systemic disease 4
Immunosuppressive agents, such as cyclophosphamide, azathioprine, methotrexate, leflunomide, and mycophenolate, may be used primarily as glucocorticoid-sparing agents 4
B-cell targeted therapies, such as rituximab, may be considered for patients with severe, refractory systemic disease 4

Treatment of B-cell lymphoma should be individualized according to histological subtype and disease stage 4
Failing to distinguish between treatment of sicca symptoms versus systemic disease should be avoided 4
Prolonged use of glucocorticoids without appropriate steroid-sparing strategies should be avoided 4
Overlooking the need for multidisciplinary care, especially for complex manifestations like neuropathy, should be avoided 5