<p>Guidelines for Sjögren’s disease (SjD) have historically been limited, particularly for managing the peripheral nervous system (PNS) manifestations of SjD. In response to this unmet need, SjD experts convened at <strong>ACR Convergence 2025</strong> to present the new Sjögren’s Foundation PNS disorders clinical practice guidelines.</p> <p><br></p> <p><em>Following this session, featured expert R. Hal Scofield, MD, was interviewed by </em>Conference Reporter <em>Medical Director Lauren Weinand, MD. Clinical perspectives from Dr Scofield on these issues are presented here.</em></p>

Most patients with SjD will experience some type of systemic manifestation. As we presented at the recent ACR Convergence 2025 meeting, the PNS manifestations of SjD can be divided most easily into mononeuropathy, polyneuropathy, and autonomic neuropathy. However, the nomenclature of PNS disorders has not been consistent, which can make evaluation and management challenging. Our Sjögren’s Foundation PNS disorders clinical practice guidelines development panel, composed of similar numbers of rheumatologists and neurologists, published a paper this year to address this issue. Everybody has to use a similar vocabulary to be able to talk about these issues in a way that makes sense between specialists, and these recommendations for aligned nomenclature of peripheral nervous system disorders were very much a collaboration between the 2 specialties.

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The actual paper that will have the new guidelines regarding the PNS manifestations of SjD is in press. Several years ago, we began to create these guidelines for SjD, as they did not exist previously. This guideline initiative was really the work of the Sjögren’s Foundation, which has been instrumental in getting everyone together to develop these guidelines. We are also currently working in earnest on guidelines for the central nervous system manifestations of SjD.

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As discussed at this year’s ACR Convergence meeting, we are following the Delphi process for the guidelines, which is recommended by the American College of Rheumatology, but these guidelines for SjD are difficult to develop because there is not a huge amount of data out there. There have not really been many successful double-blind, randomized, placebo-controlled, phase 3 trials on how to manage SjD in general, and there are no US Food and Drug Administration (FDA)–approved disease-modifying therapies. However, multiple drugs are being studied.

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Thus, much of our management strategy as described in our upcoming guidelines is based on extrapolating what is known about complications in other diseases. For instance, mononeuropathy in SjD often starts with either facial or trigeminal nerve involvement. And the most important things to include in the evaluation are the history and physical examination to differentiate whether it is a fifth or a seventh cranial nerve dysfunction, and then we go from there, with much of the data borrowed from the neurologic literature about trigeminal neuropathies.

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Another mononeuropathy that we considered when developing the guidelines was one that causes hand pain, numbness, and weakness. Again, an evaluation that includes a history and physical examination plus, maybe, electrodiagnostic studies is important to differentiate ulnar neuropathy from median neuropathy, the latter of which is also a manifestation of carpal tunnel syndrome. Additionally, it is important to keep in mind the possibility of amyloidosis in these patients.

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The guidelines are also including a discussion of multifocal neuropathy, which mainly causes pain but could also produce motor and sensory loss, as well as systemic features such as weight loss and fever. However, the most common polyneuropathy in SjD is a distal peripheral sensory neuropathy. It is very common and affects the distal small fibers, so nerve conduction velocity studies and electromyography are not very useful in this condition. The nerves are normal, which makes diagnosis difficult. And again, the history and physical examination are most important. If we have findings that are consistent with small fiber neuropathy and loss of distal sensation, then the guidelines are going to say that physicians should consider a skin biopsy in which the nerve fiber density is measured by a pathologist. If amyloidosis is a possibility, then a biopsy for amyloid may be needed. Serum cryoglobulins should also be assessed in this situation to determine whether it might be vasculitis. However, all these things are somewhat logistically difficult, and we talked about that during our presentation.

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PNS manifestations are common in SjD, and patients should be routinely, longitudinally monitored. That will mostly be accomplished through a history and physical examination to determine if they might have some of these neurologic manifestations, and then it is just a matter of deciding how to work them up.