Neurologic complications of primary Sjögren's syndrome

• Published in: Medicine (Baltimore) Vol. 61; no. 4; pp. 247 - 257
• Main Authors: Alexander, E L, Provost, T T, Stevens, M B, Alexander, G E
• Format: Journal Article
• Language: English
• Published: United States 01-07-1982
• Subjects: Adult; Antibodies, Antinuclear - analysis; Brain - diagnostic imaging; Central Nervous System Diseases - etiology; Female; Humans; Male; Middle Aged; Nervous System Diseases - etiology; Peripheral Nervous System Diseases - etiology; Raynaud Disease - etiology; Sjogren's Syndrome - blood; Sjogren's Syndrome - complications; Tomography, X-Ray Computed; Vasculitis - etiology; Xerophthalmia - etiology; Xerostomia - etiology
• ISSN: 0025-7974
• DOI: 10.1097/00005792-198207000-00004

Although peripheral nervous system disease has been well documented in Sjögren's syndrome (SS), central nervous system (CNS) involvement is considered distinctly uncommon. Sixteen patients with primary SS and CNS disorders not attributable to other causes were the subjects of this study. Cerebral manifestations, both focal and diffuse, as well as spinal cord disease, were observed. Peripheral vasculitis occurred in 12 patients (75%), 83% of whom had anti-Ro(SSA) antibodies. The high proportion of patients with concomitant peripheral vasculitis, and the observed association with antibodies to the Ro(SSA) antigen system which, in other studies, has been linked to vasculitis in SS, suggest that an immune vasculopathy may play a role in the pathogenesis of the CNS disease of SS.