ANTI-RO(SS-A) AUTOANTIBODIES IN CENTRAL-NERVOUS-SYSTEM DISEASE-ASSOCIATED WITH SJOGRANS SYNDROME (CNS-SS) - CLINICAL, NEUROIMAGING, AND ANGIOGRAPHIC CORRELATES

Objective: To examine in Sjogren's syndrome (SS) the interrelationship between the presence of the anti-Ro(SS-A) antibody response and (1) c oncomitant presence and type (ie, focal or nonfocal) of CNS disease (C NS-SS), (2) cross-sectional brain MRI or CT, and (3) abnormal cerebral angiography. Methods: Neurologic, neuroimaging, and angiographic feat ures of CNS-SS patients were correlated with the presence of precipita ting anti-Ro(SS-A) autoantibodies detected by gel double-immunodiffusi on or quantitative ELISA, which detects antibodies directed against th e 60-kd peptide. Statistical analyses were per formed using Fisher's e xact test (two-tailed) with Haldane's adjustment and odds ratio with C ornfield 95% confidence intervals. Results: Precipitating antibodies a gainst the Ro(SS-A) antigen, determined by gel double-immunodiffusion, were present in an increased frequency in CNS-SS patients with (1) do cumented clinical CNS disease, (2) focal clinical CNS manifestations a nd serious complications, (3) large regions of increased signal intens ity, consistent with ischemia/infarcts on brain MRI scans or regions o f decreased attenuation consistent with infarcts on CT, and (4) abnorm al cerebral angiograms consistent with small-vessel angiitis. Finally, the anti-Ro(SS-A) antibody response in CNS was directed against the 6 0-kd peptide specificity, determined by ELISA. Conclusions: Clinical, neuroimaging (cerebral CT), and angiographic observation suggest that a subset of anti-Ro(SS-A) antibody-positive, in contrast with -negativ e, CNS-SS patients have more serious and extensive CNS disease, some w ith frank cerebral angiopathy. Anti-Ro(SS-A) antibodies are postulated to play a role in mediating or potentiating vascular injury in CNS-SS .