What is the treatment approach for a patient's syndrome?

Treatment of Sjögren's Syndrome

For patients with Sjögren's syndrome, treatment should be stratified based on disease activity using the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI), with systemic immunosuppression reserved only for those with active systemic disease after careful organ-specific evaluation. 1

Initial Assessment and Baseline Evaluation

• Measure salivary gland function using whole salivary flow tests before initiating any treatment for oral dryness, as subjective symptoms often do not correlate with objective glandular function 1
• Assess disease activity using the ESSDAI scoring system to quantify severity and guide treatment intensity 1, 2
• Obtain baseline chest radiography and complete pulmonary function tests even in asymptomatic patients to identify subclinical interstitial lung disease, which occurs frequently in anti-SS-A (Ro) positive patients 2
• Screen for systemic involvement including cardiovascular, pulmonary, renal, neurologic, and hematologic manifestations before determining treatment approach 1

Treatment Algorithm Based on Disease Severity

For Oral Dryness (Sicca Symptoms)

Patients with mild glandular dysfunction:

• Start with non-pharmacological glandular stimulation using sugar-free acidic candies, lozenges, xylitol, or sugar-free chewing gum as first-line therapy 1
• These mechanical and gustatory stimulants work because residual glandular function can still be activated 1

Patients with severe glandular dysfunction:

• Use saliva substitutes when salivary flow measurements indicate minimal residual function 1
• Pharmacological stimulation is ineffective when glands cannot respond 1

For Systemic Disease Management

Low disease activity (ESSDAI 1-4):

• Consider hydroxychloroquine for fatigue and arthralgias 2
• Manage sicca symptoms with topical therapies including artificial tears and saliva substitutes 2

Moderate disease activity (ESSDAI 5-13):

• Initiate glucocorticoids at minimum effective dose (typically 0.5 mg/kg prednisone equivalent) 2
• Add immunosuppressive agents (azathioprine, methotrexate, or mycophenolate mofetil) for steroid-sparing effect 1

Severe systemic disease (see Table 2 in guidelines for organ-specific criteria):

• Use intensive immunosuppressive therapy following a two-stage sequential regimen: induction of remission followed by maintenance therapy 1
• For severe manifestations including demyelinating disease with motor deficit, cerebral vasculitis, myelitis, meningoencephalitis, severe hemolytic anemia (<80 g/dL), or severe thrombocytopenia (<50,000/mm³), consider rituximab, intravenous immunoglobulins, or high-dose methylprednisolone 1

Organ-Specific Treatment Protocols

Interstitial lung disease:

• First-line treatment is mycophenolate mofetil or azathioprine plus moderate-dose corticosteroids 2
• Add nintedanib as second-line maintenance for progressive fibrotic ILD 2
• Repeat pulmonary function tests every 6-12 months in patients with baseline abnormalities or respiratory symptoms 2

Peripheral neuropathy:

• Consider immunosuppressive therapy for chronic inflammatory demyelinating polyradiculopathy or other severe neuropathic manifestations 1

Monitoring and Follow-Up

• Define therapeutic response as ≥3 point reduction in global ESSDAI score 2
• Reassess ESSDAI at regular intervals to guide treatment adjustments 2
• Monitor for lymphadenopathy, fevers, and night sweats at each visit, as 2-5% of patients develop lymphoma 2
• Perform high-resolution CT if respiratory symptoms develop or pulmonary function tests become abnormal 2

Critical Pitfalls to Avoid

• Do not use systemic immunosuppression for isolated sicca symptoms without evidence of active systemic disease, as these therapies carry significant risks 1
• Avoid treating based solely on subjective dryness complaints without objective measurement of salivary gland function, as environmental and stress factors can influence symptoms 1
• Do not delay treatment of severe organ involvement (such as vasculitis or severe cytopenias), as damage may become irreversible 1
• Rule out candidiasis and burning mouth syndrome before attributing oral symptoms to Sjögren's syndrome 1