Aseptic meningitis as an initial manifestation of primary Sjögren's syndrome.

Aseptic meningitis is a rare life-threatening complication of primary Sjögren's syndrome (pSS) and its characteristics and prognosis remain unknown. We present our case of aseptic meningitis associated with pSS and reviewed the published literature to elucidate their characteristics and prognosis. An 84-year-old man was admitted to our hospital for fever and disturbance of consciousness. Acute aseptic meningitis was diagnosed based on the results for cerebrospinal fluid and head imaging tests. As an etiological investigation for his aseptic meningitis, serum anti-SSA and anti-SSB antibodies were found to be positive and the biopsy specimen of his labial salivary gland revealed a lymphocytic sialadenitis, confirming a diagnosis with pSS. Treatment with moderate-dose glucocorticoid completely improved his aseptic meningitis. Relapse of the disease was not observed during his clinical course over 12 months. Our present case and literature review suggest that aseptic meningitis can be an initial manifestation of pSS and treatable by immunosuppressive therapy. Thus, early recognition and treatment initiation are critical to prevent irreversible damage of central nerve system in pSS-associated aseptic meningitis. In aseptic meningitis of unknown origin, pSS should be included in differential diagnoses, and testing for serum anti-SSA and anti-SSB antibodies may be useful as an initial screening.