In Sjögren syndrome presenting with axonal peripheral neuropathy and active systemic disease, initial glucocorticoid therapy does not always achieve the required response. A defined next-step protocol applies in that situation.
This protocol is for Sjögren syndrome with axonal peripheral neuropathy in the setting of active systemic disease. Systemic involvement is evaluated and stratified using the ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index), and management is tailored to organ-specific severity.
The initial approach for axonal peripheral neuropathy in this setting involved glucocorticoids. Escalation to this protocol is triggered when that therapy does not achieve its required target:
Target not met: ≥3-point reduction in global ESSDAI scoreWhen glucocorticoids fail to reach the response threshold, the protocol moves to immunosuppressive therapy as the next treatment step. Several agents within this class are considered; the full selection criteria and management guidance are in the complete protocol.
The therapeutic goal at this stage remains:
≥3-point reduction in global ESSDAI scoreDOI: 10.1136/annrheumdis-2019-216114
Neuropathy (including ganglionopathy and polyradiculopathies) with severe motor deficit/ataxia; cryoglobulinemic-related multineuritis.
Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
The lack of head-to-head studies comparing the efficacy and safety profile of immunosuppressive agents in primary SjS-2002 (leflunomide, methotrexate, azathioprine, mycophenolate, cyclophosphamide) does not permit a recommendation on the use of one agent over another.
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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