Sjögren’s disease, the second most common autoimmune rheumatic disorder, can affect any body organ or system. Sjögren’s PNS manifestations occur frequently and can encompass mononeuropathy, polyneuropathy, and autonomic neuropathy.

The Sjögren’s Foundation convened a Topic Review Group (TRG) to conduct a systematic review and develop evidence-based guidelines based on the methodology of the American College of Rheumatology (ACR)¹ and the Sjögren’s Syndrome Foundation,² 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.^3-5  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.

The clinical practice guidelines include recommendations for the evaluation and management of peripheral nervous system manifestations in patients with Sjögren’s disease, covering mononeuropathy (cranial neuropathies, peripheral nerve neuropathies),  polyneuropathy (Large Fiber Neuropathy, Small Fiber Neuropathy (SFN), Demyelinating Polyradiculoneuropathy, Ganglionopathy (neuronopathy), Vasculitic Neuropathy), and autonomic neuropathy (autonomic ganglionopathy,  orthostatic intolerance, and gastrointestinal dysmotility).