Treatment of Sjögren's Syndrome Flare
The treatment of a Sjögren's syndrome flare should follow a logical sequence starting with management of the central symptoms (dryness, fatigue, pain) followed by targeted therapy for systemic manifestations based on organ-specific severity. 1
General Approach to Management
- A multidisciplinary approach involving specialists in autoimmune diseases as coordinators is essential, with shared decision-making between the patient and healthcare team 1
- Treatment should be tailored to the specific manifestations present during the flare, with different approaches for sicca symptoms versus systemic disease 1
Management of Sicca Symptoms (Dryness)
Oral Dryness
- For mild salivary gland dysfunction: non-pharmacological stimulation (sugar-free gum, frequent sips of water) 1
- For moderate dysfunction: pharmacological stimulation with muscarinic agonists (pilocarpine 5mg three times daily or cevimeline) 1, 2
- For severe dysfunction: saliva substitutes 1
- Pilocarpine has demonstrated statistically significant improvement in salivary flow compared to artificial saliva (p<0.001) 2
Ocular Dryness
- First-line: artificial tears and ocular gels/ointments 1
- For refractory/severe cases: topical immunosuppressive-containing drops and autologous serum eye drops 1
- Common side effects of pilocarpine include sialorrhea and nausea 2
Management of Constitutional Symptoms
Fatigue and Pain
- Evaluate for concomitant conditions that may contribute to fatigue/pain 1
- Score severity using specific tools 1
- For musculoskeletal pain: consider analgesics or other pain-modifying agents, weighing benefits against side effects 1
- Physical exercise may help manage mild systemic symptoms like fatigue 3
Management of Systemic Disease Manifestations
General Approach
- Treatment should be tailored to organ-specific severity using ESSDAI definitions 1
- Follow a sequential approach based on severity 1:
- Glucocorticoids (minimum dose and duration necessary)
- Immunosuppressive agents as steroid-sparing options
- Biological therapies for refractory cases
Specific Medications for Systemic Disease
Glucocorticoids
- Should be used at the minimum dose and for the shortest time necessary to control active systemic disease 1
- Particularly useful for acute systemic manifestations 3
Immunosuppressive Agents
- Used primarily as glucocorticoid-sparing agents 1
- Options include cyclophosphamide, azathioprine, methotrexate, leflunomide, and mycophenolate 1
- No strong evidence supports choosing one agent over another 1
Biological Therapies
- B-cell targeted therapies (particularly rituximab) may be considered for patients with severe, refractory systemic disease 1, 4
- Rituximab has shown moderate effectiveness in reducing hyposalivation in Sjögren's patients 4
- Anti-TNF agents have not demonstrated significant benefit in Sjögren's syndrome 5
Neuropathic Manifestations
- For neuropathy associated with Sjögren's: treatment follows the sequential approach (glucocorticoids → immunosuppressants → biologics) 6
- Symptomatic treatment of neuropathic pain with agents such as pregabalin, gabapentin, or duloxetine is recommended 6
Special Considerations
- Treatment of B-cell lymphoma (a potential complication in 2-5% of patients) should be individualized according to histological subtype and disease stage 1
- Hydroxychloroquine has been used empirically but shows limited efficacy as a disease-modifying agent 5
- Monitor for adverse effects, particularly with pharmacological therapies 4
Common Pitfalls to Avoid
- Failing to distinguish between treatment of sicca symptoms versus systemic disease 1
- Using systemic therapies for purely sicca symptoms without systemic manifestations 3
- Prolonged use of glucocorticoids without appropriate steroid-sparing strategies 1
- Overlooking the need for multidisciplinary care, especially for complex manifestations like neuropathy 6