Background/Purpose: Peripheral Nervous System (PNS) manifestations occur frequently in Sjögren’s disease and can encompass mononeuropathy, polyneuropathy, and autonomic neuropathy. Guidance is needed for rheumatologists, neurologists, and Sjögren’s patients on PNS diagnosis, management and treatment. The objective of this work is to provide evidence-based clinical practice guidelines for peripheral nervous system (PNS) manifestations in patients with Sjögren’s disease.

Methods: The Sjögren’s Foundation convened a Topic Review Group (TRG) comprised of rheumatologists & neurologists to conduct a systematic review and develop evidence-based guidelines based on the methodology of the American College of Rheumatology (ACR) and the Sjögren’s Foundation, and adherent to the principles of the American Society of Clinical Oncology, Grading of Recommendations Assessment, Development and Evaluation, and the US Preventive Services Taskforce. A guideline methodologist was engaged to ensure the most up-to-date methodology was followed. The TRG defined PICO questions, inclusion/exclusion criteria, and endpoints. PubMed was searched for English-language articles from January 1, 1990, to May 1, 2023. Title and abstract screening, full-text screening, data extraction, and quality assessment were done in duplicate. The strength of each recommendation was rated based on the available evidence and the confidence level that the recommendation offers the best current guidance for practice. A Delphi Consensus Expert Panel voted on its extent of agreement and provided commentary on the recommendations for TRG review.

Results: Seven studies met inclusion criteria—five case series and two prospective cohort studies without a comparison group. Twenty-eight Best Practices and twenty-three Recommendations were crafted. Limited study availability and low evidence quality led to all treatment recommendations being rated as weak. Given the dearth of evidence on work-up in a Sjögren’s-specific population, the TRG adopted a best-practices approach, which is care that is deemed optimal based on a prevailing standard, for evaluation of PNS manifestations in Sjögren’s patients.

Conclusion: The clinical practice guidelines include best practices and recommendations for evaluating and managing PNS manifestations in patients with Sjögren’s disease. These cover mononeuropathy (cranial neuropathies, peripheral nerve neuropathies),  polyneuropathy (large fiber neuropathy, small fiber neuropathy, demyelinating polyradiculoneuropathy, ganglionopathy (neuronopathy), vasculitic neuropathy) and autonomic neuropathy (autonomic ganglionopathy, orthostatic intolerance, and gastrointestinal dysmotility).

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/evidence-based-guidelines-for-sjogrens-disease-peripheral-nervous-system-manifestations/