Primary Sjögren’s syndrome (pSS) rarely involves the CNS, including cases of aseptic meningitis (AM). We report the case of a 59-year-old woman who presented with headache, fever, and nuchal rigidity. CSF analysis revealed pleocytosis, elevated protein, and decreased glucose levels. Both serum and CSF anti-Sjögren’s syndrome antigen A (anti-SS-A) antibody tests were positive, with an elevated CSF anti-SS-A antibody index. Salivary gland biopsy confirmed the diagnosis of pSS. The patient improved following steroid pulse therapy. Reduced CSF glucose levels in combination with positive CSF anti-SS-A antibody tests may serve as supportive clues for pSS-associated AM. Evaluation of autoimmune features and CSF anti-SS-A antibodies may aid in the diagnosis of AM of unclear etiology.
Ueno et al. (Thu,) studied this question.