Treatment of Extraglandular Sjögren's Syndrome

Initial Assessment and Treatment Stratification

The European League Against Rheumatism (EULAR) recommends assessing disease activity using the ESSDAI scoring system to quantify severity, with treatment decisions based on organ-specific severity 5, 4
For patients with low disease activity (ESSDAI 1-4), observation or hydroxychloroquine may be considered 4
For patients with moderate disease activity (ESSDAI 5-13), initiation of glucocorticoids with steroid-sparing agents is recommended 4
For patients with high disease activity (ESSDAI ≥14), aggressive immunosuppression is required 5, 3

For interstitial lung disease (ILD) with symptomatic or moderate-severe impairment, first-line treatment with mycophenolate mofetil (MMF) or azathioprine as steroid-sparing agents combined with moderate-dose oral corticosteroids is recommended 6, 7
For progressive fibrotic ILD, addition of nintedanib as second-line maintenance therapy is suggested 7
For cryoglobulinemic vasculitis, first-line treatment with moderate-to-high dose glucocorticoids (0.5-1 mg/kg prednisone equivalent) is recommended 1, 8
For peripheral neuropathy, treatment with glucocorticoids plus azathioprine or mycophenolate is recommended for progressive or motor involvement 5, 6

The American College of Rheumatology recommends using glucocorticoids as first-line therapy, with dosing strategy based on disease severity 2, 8
For severe disease, high-dose IV methylprednisolone (1g daily for 3-5 days) may be used 7
Steroid-sparing immunosuppressive agents, such as mycophenolate mofetil or azathioprine, should be initiated early to facilitate glucocorticoid tapering 2, 8

Rituximab may be considered for severe, refractory systemic disease, particularly for cryoglobulinemic vasculitis, severe peripheral neuropathy, or refractory cytopenias 1, 8

For low-grade lymphoma (MALT, marginal zone, SLL, LPL), watchful waiting or radiotherapy ± chemotherapy may be considered 1, 8
For high-grade lymphoma, standard rituximab-based chemotherapy regimens should be used 1

The EULAR recommends defining therapeutic response as reduction of ≥3 points in global ESSDAI score 5, 4
Regular monitoring of ESSDAI score, organ-specific parameters, and surveillance for lymphoma development is essential 3, 4, 10