This protocol covers patients with Sjögren syndrome who have axonal peripheral neuropathy alongside active systemic disease — a presentation that signals significant neurological and systemic involvement and informs how aggressively treatment is structured.
Peripheral neuropathy in Sjögren syndrome encompasses axonal forms as well as ganglionopathy and polyradiculopathies. Presentations with severe motor deficit or ataxia represent high-severity neurological involvement.
Treatment of the systemic disease is tailored to organ-specific severity, assessed according to the ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index) definitions.
The structured regimen involves glucocorticoid therapy, with the form and intensity adapted to the severity of systemic involvement. The protocol distinguishes between moderate and severe presentations.
The primary therapeutic target is a reduction of 3 or more points in the global ESSDAI score, indicating meaningful control of systemic disease activity.
DOI: 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.
GCs should be used at the minimum dose and length of time necessary to control active systemic disease.
GC (recommended dose in mg/kg/day); short-term course whenever possible; consider methylprednisolone pulses in severe cases.
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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