Evidence-based Guidelines for Sjögren’s Disease Peripheral Nervous System Manifestations
George Sarka1, Anahita Deboo2, Robert Fox3, Matthew Baker4, Nancy Carteron5, Stamatina Danielides6, Julie Frantsve-Hawley7, Brent Goodman8, Katherine Hammitt9, Jennifer King 10, Matt Makara11, Ghaith Noaiseh12, Pantelis Pavlakis13, Steven Mandel14, R. Hal Scofield15, Arun Varadhachary16, Daniel Wallace17, Steven Carsons18
1CSUN/UCLA/CSMC, 2Temple University School of Medicine - Neurology, 3Rheumatology, La Jolla and Scripps, 4Stanford, 5University of California, Berkeley, 6Inova Fairfax, 7Sjögren’s Foundation, 8Dept of Neuro /Mayo Clinic, 9Sjogren's Foundation, 10UCLA, 11Sjögren's Foundation, 12University of Kansas, 13Hospital for Special Surgery, 14Hofstra Northwell School of Medicine, 15OMRF, 16Washington University in St Louis, 17Cedars-Sinai Medical Center, 18NYU Langone-Winthrop
Objective:
To provide evidence-based clinical practice guidelines for peripheral nervous system (PNS) manifestations in patients with Sjögren’s disease.
Background:

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.

Design/Methods:

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.

Results:
Seven studies met inclusion criteria—five case series and two prospective cohort studies without a comparison group. 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 work-up of PNS manifestations in Sjögren’s patients.6   
Conclusions:

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). 

10.1212/WNL.0000000000205708