New guidelines to manage peripheral neuropathy related to Sjögren disease have been developed by a multidisciplinary team of physicians from across medicine.
The guidelines will provide an evidence-based resource for the assessment, diagnosis, and treatment of various peripheral neuropathies related to the disorder.
Up until now, the field has been "haphazard and chaotic," lead author George Sarka, MD, DrPH, MPH, director of the CME Committee for MemorialCare, Saddleback Medical Center, Laguna Hills, California, and member of the Sjögren's Foundation PNS Guidelines Topic Review Group (TRG), told Medscape Medical News.
Sarka discussed the initiative on April 17 at the American Academy of Neurology (AAN) 2024 Annual Meeting.
Severe, Complex Illness
Sjögren disease is the second most common autoimmune rheumatic disorder after rheumatoid arthritis, affecting an estimated 4 million Americans. Women make up most of the patient population at a ratio of 9:1.
The condition typically affects the mucous membranes and moisture-secreting glands of the eyes and mouth, resulting in decreased tears and saliva. But peripheral nervous system (PNS) manifestations often precede these symptoms and can occur in up to 60% of Sjögren disease cases.
"Traditionally, Sjögren's was looked at as a dry eye and dry mouth disease, but we realize now that it's so much broader than that," said Sarka. "It's a severe, systemic, and complex illness that can affect any body organ or system, and the nervous system is frequently affected."
PNS manifestations cause more than mere discomfort; they can lead to diagnostic and management challenges, costly treatments, and diminished quality of life.
Getting a proper diagnosis goes a long way toward improving the quality of life for these patients, Steven Mandel, MD, clinical professor of neurology at the Zucker School of Medicine at Hofstra-Northwell, adjunct clinical professor of medicine at NY Medical College, New York City, and member of the TRG, told Medscape Medical News.
The problem is, doctors don't always think an autoimmune disorder is causing the symptoms, said Sarka. "There's an old adage in neurology that if you don't think about it, you're going to miss it; you have to ask, and that's what we're trying to get people to do."
The condition often accompanies other immune system disorders such as rheumatoid arthritis and lupus. But as patients are referred back and forth between ophthalmologists, rheumatologists, and neurologists, the condition is often missed. "It could be 4 or 5 years before a definitive diagnosis of Sjögren's is made," said Sarka.
He believes the education system is partly to blame. "Medical schools have been very deficient in teaching people about recognizing Sjögren's disease."
That leaves many physicians at a loss about "what to do with these patients when they walk in the door," said Mandel. "They don't know how to manage them; they don't know how to diagnose them; and they don't know how to treat them."
Developing guidelines with multispecialty collaboration was "absolutely critical" in addressing this knowledge gap, Mandel added. That process involved "a very rigorous and transparent methodology so that it would be accepted by all the professionals involved in Sjögren's," he said.
The process took 3 years and involved amassing and grading the evidence, getting consensus from committee members, developing recommendations, and getting feedback and external review.
Scant Evidence
An early literature search revealed very little evidence on PNS manifestations in patients with Sjögren disease, so the guideline committee "leaned very heavily on expert opinion" to develop recommendations, Kathy Hammitt, MA, vice president of Medical and Scientific Affairs, Sjögren's Foundation, told Medscape Medical News.
The literature search also showed different terms are used to describe PNS, "which is where the chaos comes in," said Sarka.
Experts from different specialties worked together to define and align nomenclature used by various specialists. They developed definitions for seven PNS categories including mononeuropathy, large fiber neuropathy, small fiber neuropathy, demyelinating polyradiculoneuropathy, ganglionopathy, vasculitis neuropathy, and autoimmune nervous system neuropathy.
The guidelines pertaining to PNS manifestations encompass a spectrum of neurologic abnormalities, including cranial neuropathies (trigeminal neuropathy or acute facial neuropathy), polyneuropathies (large fiber neuropathy, small fiber neuropathy, demyelinating polyradiculoneuropathy, vasculitis neuropathy, or ganglionopathy), and autonomic nervous system (ANS) neuropathies (postural tachycardia, orthostatic hypotension, or autonomic dysfunction).
Key Steps
The guidelines address two key steps for each PNS manifestation — the workup and evaluation of patients with suspected ANS manifestation including standard evaluations, diagnostic tests, and treatment. The experts developed 31 best practices for diagnosis and workup and 20 treatment recommendations.
Initial assessment of potential ANS involvement includes asking patients about orthostatic postural lightheadedness and difficulties with digestion, urination, sweating, and sexual function.
Treatment of autoimmune diseases typically focuses on relieving symptoms and can include steroids, the anticonvulsant gabapentin, the monoclonal antibody rituximab, and intravenous immunoglobulin. "The type of neuropathy will mandate or suggest certain therapies over others," said Sarka, adding a patient can have more than one neuropathy.
Therapeutics for Sjögren disease is another example of an area that has been "very haphazard," he added.
The guidelines are aimed not just at specialists but also at general practitioners who treat many of these patients. But Hammitt emphasized that neurologists can be "instrumental" in identifying Sjögren disease in patients with PNS symptoms.
"Our hope is that specialists — in this case, neurologists — will recognize the potential for this condition in their PNS patients and ensure referral to a rheumatologist or knowledgeable family practitioner to manage overall care."
The committee will soon submit its manuscript to the AAN for publication.
"Once published, we will have a robust dissemination strategy to ensure that providers, patients, and policymakers are aware of, and use, this very valuable resource," said Hammitt.
No conflicts of interest were reported.