AUTHOR=Afzali Ali M. , Moog Philipp , Kalluri Sudhakar Reddy , Hofauer Benedikt , Knopf Andreas , Kirschke Jan Stefan , Hemmer Bernhard , Berthele Achim 

TITLE=CNS demyelinating events in primary Sjögren's syndrome: A single-center case series on the clinical phenotype

JOURNAL=Frontiers in Neurology

VOLUME=Volume 14 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2023.1128315

DOI=10.3389/fneur.2023.1128315

ISSN=1664-2295

ABSTRACT=Objective: To assess the prevalence, clinical characteristics and therapeutic outcome of central nervous system (CNS) demyelinating disease in a large cohort of primary Sjögren's syndrome (pSS). 
Methods: Explorative cross-sectional study of patients with pSS seen in the Departments of Rheumatology, Otorhinolaryngology or Neurology of a tertiary university center between January 2015 and September 2021.
Results: In a cohort of 194 pSS patients, twenty-two patients had an affection of the CNS. In this CNS group, nineteen patients had a lesion pattern suggestive of demyelination. While there were no obvious differences in the patients’ epidemiological disposition or rate of other extraglandular manifestations, the CNS group differed from the remaining pSS patients by having less glandular manifestations, but a higher seroprevalence for anti-SSA-/Ro-antibodies. Notably, patients with CNS manifestations were often diagnosed with multiple sclerosis (MS) and treated as such, although age and disease course were atypical of MS. Many first-line MS agents were ineffective in these “MS look-alikes“, however, the disease course was benign with B-cell depleting agents.
Conclusion: Neurological symptoms of pSS are common and clinically manifest mainly as myelitis or optic neuritis. Notably, in the CNS, the pSS phenotype can overlap with MS. The prevailing disease is crucial since it has a major impact on the long-term clinical outcome and the choice of disease-modifying agents. Although our observations neither confirm pSS as the more appropriate diagnosis nor rule out simple comorbidity, physicians should consider pSS in the extended diagnostic workup of CNS autoimmune diseases.