Sjögren Syndrome with Systemic Glandular Involvement and Parotid Swelling: What to Do When Rituximab Did Not Achieve an ESSDAI Response
This protocol addresses active Sjögren syndrome with systemic glandular involvement — including parotid or salivary gland swelling — in patients whose previous biologic course with rituximab did not produce the required reduction in disease activity. A structured next-line approach is defined for this scenario.
Sjögren syndrome with systemic glandular involvement (parotid or salivary gland swelling) and active systemic disease, defined by a clinESSDAI score ≥1. Systemic disease refers to involvement that affects or has affected any of the organs or systems included in the clinESSDAI score.
A course of rituximab was completed. The required therapeutic target — a reduction of ≥3 points in the global ESSDAI score — was not achieved. This protocol defines the next step following that failure.
References
- The systemic organ-specific therapeutic approach may follow, as a general rule, the sequential (or combined) use of GCs, immunosuppressive agents and biologics.
- Systemic disease: Disease involvement that affects or has affected any of the organs/systems included in the clinESSDAI score.
- With respect to the definition of the therapeutic response in systemic SjS, the TF recommends using a reduction of ≥3 points in the global ESSDAI score.
- BLM: belimumab; 10 mg/kg (0, 2 and 4 weeks and then every 4 weeks).
- Of five patients previously refractory to rituximab, belimumab was effective in 3 (60%).