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 aetiological investigation for his aseptic meningitis, serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen B antibodies were found to be positive, and the biopsy specimen of his labial salivary gland revealed lymphocytic sialadenitis, confirming a diagnosis of 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 be treatable by immunosuppressive therapy. Thus, early recognition and treatment initiation are critical to prevent the irreversible damage of central nervous system in pSS-associated aseptic meningitis. In aseptic meningitis of unknown origin, pSS should be included in differential diagnoses, and testing for serum anti-Sjögren's-syndrome-related antigen A and anti-Sjögren's-syndrome-related antigen A antibodies may be useful as an initial screening.

Higashida-Konishi M ，Akiyama M ，Shimada T ，Hama S ，Oshige T ，Izumi K ，Oshima H ，Okano Y ... -  《-》

Recurrent aseptic meningitis: A rare clinical presentation of Sjogren's syndrome.

Basheer A ，Khan EA ，Hashmi T ，Khan RA ，Ahmad A ... -  《JOURNAL OF THE PAKISTAN MEDICAL ASSOCIATION》

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?

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.

Kurotaki K ，Fujita M ，Aizawa T ，Tsugawa K ，Tanaka H ... -  《-》

Acute encephalitis in primary Sjögren's syndrome: A case report and literature review.

Acute encephalitis is an extremely rare condition in primary Sjogren's syndrome (pSS), and its characteristics and prognosis remain unclear. Here, we report the case of pSS presented with acute encephalitis. She was admitted to our hospital for acute disturbance of consciousness. Acute encephalitis was diagnosed based on the results of the cerebrospinal fluid test (the increase of leucocyte counts, proteins, and interleukin-6 levels), magnetic resonance imaging, and single-photon emission computed tomography with 99mTc. The infectious aetiologies and underlying malignancies were excluded. Serum anti-Sjogren's syndrome-related antigen A autoantibody was positive with extremely high titre. The biopsy specimen of her labial salivary gland revealed a focal lymphocytic sialadenitis with a score of grade 4 in the Greenspan grade. She also developed diffuse alveolar haemorrhage during the clinical course. She was diagnosed with pSS complicated with acute encephalitis followed by diffuse alveolar haemorrhage and successfully treated with pulse steroids, high dose of prednisolone and intravenous cyclophosphamide. Our present case and literature review suggest that acute encephalitis associated with pSS can be treatable with the immunosuppressive therapy, and thus early recognition and treatment initiation are important for this life-threatening condition. Thus, pSS should be included in the differential diagnosis of unexplained encephalitis. Notably, our case characteristically showed diffuse alveolar haemorrhage, adding new insights into the pathogenesis of acute encephalitis associated with pSS that capillaritis might be the underlying cause of this condition.

Higashida-Konishi M ，Akiyama M ，Shimada T ，Hama S ，Takei H ，Izumi K ，Oshima H ，Okano Y ... -  《-》

Anti-Sjögren's-syndrome-related antigen A autoantibodies (Anti-SSA antibody) and meningoencephalitis: Sjögren's syndrome waiting to be unveiled? A case series and review of literature.

Mittal GK ，Mittal S ，Kaur H ，Stephen SR ，Sekhar S ，Sureshbabu S ，Sandhya P ... -  《-》