Sjögren Syndrome with Cutaneous Annular Erythema and Active Systemic Disease: What to Do When Glucocorticoids Have Not Achieved Disease Control
This protocol addresses patients with Sjögren syndrome presenting with cutaneous annular erythema in the context of active systemic disease, where a prior glucocorticoid regimen has not achieved the defined treatment target.
Clinical Scenario
Sjögren syndrome with cutaneous annular erythema as part of active systemic involvement. Systemic treatment follows the organ-specific severity of disease as defined by the ESSDAI, with management guided sequentially through glucocorticoids, immunosuppressive agents, and biologics.
Previous Treatment Did Not Achieve Target
When a glucocorticoid regimen does not produce a reduction of ≥3 points in the global ESSDAI score, escalation to a further step is indicated. This protocol defines the next-line approach after that failure.
Next-Step Treatment Approach
An immunosuppressive agent is the basis of the next-line strategy in this scenario. The full regimen, sequencing, and monitoring guidance are detailed in the structured protocol.
Treatment target: ESSDAI reduction ≥3 points
References
DOI: 10.1136/annrheumdis-2019-216114
The systemic organ-specific therapeutic approach may follow, as a general rule, the sequential (or combined) use of GCs, immunosuppressive agents and biologics.
Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
ID: immunosuppressive agents, no head-to-head comparisons.
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.
View source ↗