Treatment of Sjögren Syndrome with Cutaneous Annular Erythema After Hydroxychloroquine Failure

In patients with active systemic Sjögren syndrome and cutaneous annular erythema where the first-line approach has not achieved the required treatment response, a defined escalation protocol applies.

Clinical Scenario

This protocol covers Sjögren syndrome with active systemic involvement and cutaneous annular erythema. The systemic therapeutic approach may follow the sequential or combined use of different treatment classes. Management is tailored to organ-specific severity using the ESSDAI definitions, with a target of a reduction of ≥3 points in the global ESSDAI score.

Previous Treatment — Insufficient Response

The preceding treatment line used hydroxychloroquine. Escalation to this protocol is triggered when that line fails to achieve a reduction of ≥3 points in the global ESSDAI score — the defined response threshold for systemic Sjögren syndrome.

Next-Line Approach

This protocol moves to glucocorticoid therapy. Whether the approach uses an oral route or an intravenous induction sequence depends on the severity of the current disease presentation. The complete structured regimen — including the full decision algorithm — is available via the link below.

Instant Access to Structured Evidence-Based Regimens

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.

GCs should be used at the minimum dose and length of time necessary to control active systemic disease, administering pulses of methylprednisolone followed by doses of 0.5 mg/kg/d or lower as induction therapy in severe presentations, and doses <0.5 mg/kg/d in moderate/less-severe presentations, with a final target of withdrawing GCs in inactive patients as soon as possible or at least trying to target a maintenance dose of 5 mg/daily or less with the aid of GC-sparing immunosuppressive agents.

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.

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