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Abstract Details

Can Sjogren's Mimic MS or be an MS+ Syndrome?
Multiple Sclerosis
P16 - Poster Session 16 (5:30 PM-6:30 PM)
9-019

To evaluate the clinical and paraclinical findings as well treatment response of a retrospectively identified group of patients who have CNS demyelinating disease as well as diagnostic evidence of Sjogren’s Syndrome.

Primary Sjogren’s Syndrome (PSS) is considered to be a rare cause of CNS demyelinating disease. It is possible that some cases are missed as its clinical hallmark of sicca is nonspecific, the SSA/SSB antibodies have limited sensitivity, and MS presents a ready default diagnosis for CNS demyelination even if some features are insufficient or irregular.
This is a chart review of 27 patients who presented with CNS demyelinating disease and were diagnosed with Sjogren’s.
All patients had sicca, 17 (63%) patients had SSA or SSB antibodies, 9 (33%) patients were diagnosed by gland biopsy, 1 by ultrasound. Nineteen (70%) were initially diagnosed as MS but CSF testing revealed >3 unique OCB’s in 8/24 (33%), 0 OCB’s in 11/24 (45%) and mirror pattern banding in 5/24 (20%). MRI’s showed typical MS lesions in 30% while 70% had atypical or nonspecific lesions. Of the 8 patients with typical MS lesions, only 2/7 (29%) also had >3 oligoclonal bands. When the treatment paradigm was directed towards Sjogren’s, 15/19 (79%) patients had meaningful improvement in neurological function. Hallmarks prompting an evaluation for Sjogren’s included these CSF and MRI irregularities in combination with sicca, systemic symptoms and poor response to MS treatments.

These patients with Sjogren’s and CNS demyelinating disease  had a constellation of clinical presentation, CSF, MRI and treatment response which differed from what would be expected in typical MS however there was no clear uniform feature to delineate the two. The potential overlap and positive response to directed therapies should interest both scientists and clinicians.

Authors/Disclosures
Armistead Williams III, MD (International MS Management Practice)
PRESENTER
Dr. Williams has nothing to disclose.