This protocol addresses patients with Sjögren syndrome who present with neutropenia active systemic disease. Systemic disease activity is characterised and graded using the ESSDAI (EULAR Sjögren's Syndrome Disease Activity Index), and treatment should be tailored to organ-specific severity according to ESSDAI definitions.
Management of neutropenia in the context of active Sjögren systemic disease involves a targeted growth factor intervention aimed at the haematological domain. The full protocol — including the specific agent, its application, and how it integrates with overall systemic care — is available in the complete structured regimen.
Success is defined as a clinically meaningful reduction in systemic disease activity: a decrease of ≥ 3 points in the global ESSDAI score.
DOI: 10.1136/annrheumdis-2019-216114
Treatment of systemic disease should be tailored to organ-specific severity using the ESSDAI definitions.
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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