Neurologic Involvement in Seronegative Sjogren's Syndrome with Positive Lip Biopsy: A Single Center Experience

Objective: Characterize the clinical and demographic features of patients with Neurologic Involvement of Sjogren’s Syndrome, in whom classical antibody testing is negative, but who meet criteria for seronegative Sjogren’s syndrome via a supportive lip biopsy and clinical criteria, per the American-European Consensus Group (AECG) criteria. Background: Sjogren’s syndrome (SS) is an autoimmune disorder classically characterized by lymphocytic infiltration and destruction of salivary and lachrymal glands resulting in dry mouth and dry eyes. Neurologic involvement of Sjogren’s Syndrome is under-recognized, especially when the initial symptoms lead to neurologic consultation prior to a formal rheumatologic diagnosis; in 25–60% of SS cases, the neurological symptoms preceded the diagnosis of SS by 2 years. Neurologic symptoms in SS commonly include peripheral neuropathy and isolated cranial neuropathy, as well as dysautonomia, cognitive dysfunction, aseptic meningitis, epilepsy, headache, and myelopathy. Additionally, hyperintense T2 lesions on brain MRI may be present and can appear similar to lesions seen in multiple sclerosis. Design/Methods: We performed a retrospective review of the electronic medical records at the University of Utah Health and queried charts of patients with a diagnosis of SS with a positive lip biopsy and seronegative antibody status who were also evaluated on at least one occasion by a Neurologist between January 2010 to October 2018. Results: Of the seronegative SS patients identified, we describe the type of neurologic involvement (central or peripheral nervous system; with or without autonomic symptoms), comorbid conditions, associated autoimmunity, and response to immunomodulatory and anti-inflammatory treatments, as well as demographic and clinical data. Conclusions: 25–60% of SS patients present for evaluation of neurologic symptoms prior to receiving a formal diagnosis of Sjogren’s Syndrome. Therefore, Neurologists should be familiar with the range of manifestations of SS to avoid misdiagnosis, especially as those patients who are seronegative for SS may suffer even longer delays in diagnosis. Disclosure: Dr. Wong has nothing to disclose. Dr. Galli has nothing to disclose. Dr. Bacharach has nothing to disclose. Dr. Klein has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Teva and Biogen Idec. Dr. Esquibel has nothing to disclose. Dr. Pace has nothing to disclose. Dr. Lebiedz-Odrobina has nothing to disclose. Dr. Rose has received research support from the National Multiple Sclerosis Society, the Guthy Jackson Foundation, the Western Institute for Biomedical Research, Teva Neuroscience and Biogen. Dr. Trump has nothing to disclose. Dr. Hull has nothing to disclose. Dr. Greenlee has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Wheeler, Trigg, and O’Donnell. Dr. Greenlee has received personal compensation in an editorial capacity for Medlink and Merck Manual, Professional and Home. Dr. Clardy has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Adivo Associates (