How central nervous system manifestations of Sjögren’s syndrome respond to immunosuppressive therapy
The prevalence of central nervous system (CNS) dysfunction in Sjögren’s syndrome patients remains a controversial topic. Researchers reviewed the records of 250 patients with primary Sjögren’s seen from 1997-2001 to identify patients with CNS dysfunction and evidence of neurologic or neuropsychiatric dysfunction not explained by other conditions such as lupus. Each patient underwent a complete examination, including measurement of salivary flow, autoantibody and other serologic testing, tests for dry eyes and salivary scintigraphy.
A total of nine patients out of 250 (4%) showed CNS manifestations of Sjögren’s and three had concomitant peripheral neuropathy. All cases were female (ages 30-78, median 50) and six of nine were Caucasian. Patterns of CNS dysfunction included aseptic meningitis, cognitive impairment, vestibular dysfunction, optic neuritis, and transverse myelitis. Diagnostic studies included MRI of the brain and/or spinal cord, SPECT brain perfusion scan and neuropsychological testing. One patient with recurrent aseptic meningitis underwent spontaneous improvement with supportive care. Of the remaining eight patients, seven reported a reduction in neurologic symptoms with high dose systemic corticosteroids followed by a worsening when steroids were tapered. These eight patients were then given additional immunosuppressive treatments with varied outcomes ranging from remission to no change in symptoms. Sustained improvement was observed only with cyclophosphamide (Cyc), azathioprine (Az), or both. The researchers concluded that CNS involvement in Sjögren’s syndrome patients may cause devastating complications but is also potentially amenable to early aggressive immunosuppressive treatment. Corticosteroids produce transient benefits but Az and/or Cyc will more likely produce sustained remission or stabilization in the majority of cases.
Study conducted by: Joshua D. Stein, New York University, New York, NY; Kimberly P. Liang, Mayo Graduate School of Medicine, Rochester, MN; Kelly V. Liang, Mayo Graduate School of Medicine, Rochester, MN; Frederick B. Vivino, Thomas Jefferson University, Philadelphia, PA.
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