Clinical Practice Guideline for Evaluation and Management of Peripheral Nervous System Manifestations in Sjögren's Disease

Abstract

Objective: Sjögren's disease is an autoimmune disorder that can impact multiple organ systems, including the peripheral nervous system (PNS). PNS manifestations, which can exist concurrently, include mononeuropathies, polyneuropathies, and autonomic nervous system neuropathies. To help patients and providers in the decision-making process, we developed an evidence-based clinical practice guideline for the evaluation and management of PNS manifestations in patients with Sjögren's disease.

Methods: A Topic Review Group, comprising experts in rheumatology, neurology, and guideline methodology, developed Patient, Intervention, Comparison, and Outcome questions and conducted a systematic review to identify the current best evidence on management of PNS manifestations of Sjögren's disease. PubMed and Embase were searched for evidence published up to July 22, 2025. Literature screening, data extraction, and critical appraisal were performed in duplicate. Six case series, one retrospective cohort, and two prospective cohort studies lacking a comparison group met the inclusion criteria.

Results: We developed an aligned nomenclature of PNS terms that can be used across disciplines, 31 good practices for evaluation of suspected PNS manifestations, and 20 evidence-based treatment recommendations, the latter of which were rated as conditional or strong based on the Grading of Recommendations Assessment, Development, and Evaluation methodology. Because of the scarcity of high-level evidence, this guideline predominantly derives from expert opinion.

Conclusion: This clinical practice guideline on PNS manifestations of Sjögren's disease provides clinicians a rigorous, evidence-based resource, developed through an expert consensus-based process, for the assessment, diagnosis, and treatment of peripheral neuropathy in patients with Sjögren's disease. Recommendations were rated as strong when the benefits significantly outweighed potential harms, creating a scenario in which most patients would prefer the advised action.