Aseptic meningitis as initial presentation of subclinical Sjögren’s syndrome: Could the cerebrospinal fluid anti-Ro/SSA and anti-La/SSB antibody system be the culprit?

Abstract

Abstract Aseptic meningitis sometimes occurs as a consequence of central nervous system (CNS) involvement in patients with primary Sjögren’s syndrome (SS), even in paediatric-onset cases. However, little information is available regarding the pathological role of CSF anti-Ro/SSA and anti-La/SSB antibodies in the CNS involvement in patients with primary SS. We experienced an 18-year-old adolescent female with a 7-year history of suspicion of subclinical SS who subsequently developed aseptic meningitis as an initial presentation of probable SS. Her CSF exhibited marked elevation of anti-Ro/SSA and anti-La/SSB antibodies. When compared to her CSF IgG/serum IgG ratio (0.0058), her CSF/serum ratios of anti-Ro/SSA and anti-La/SSB antibody titres were higher (0.448 and 0.068, respectively; these were 77.5 and 11.7 times higher than that of IgG, respectively), suggesting that regional production of these antibodies was attributable, at least partly, to the development of meningitis. After the initiation of prednisolone treatment, her clinical manifestations promptly subsided. Since the clinical and pathological roles of the Ro/SSA antibody system in several autoimmune conditions have been postulated, our clinical observation may add novel insight to this theory.