Anti-Ro(SS-A) autoantibodies in central nervous system disease associated with Sjögren's syndrome (CNS-SS): clinical, neuroimaging, and angiographic correlates.
OBJECTIVE
To examine in Sjögren's syndrome (SS) the interrelationship between the presence of the anti-Ro(SS-A) antibody response and (1) concomitant presence and type (ie, focal or nonfocal) of CNS disease (CNS-SS), (2) cross-sectional brain MRI or CT, and (3) abnormal cerebral angiography.


METHODS
Neurologic, neuroimaging, and angiographic features of CNS-SS patients were correlated with the presence of precipitating anti-Ro(SS-A) autoantibodies detected by gel double-immunodiffusion or quantitative ELISA, which detects antibodies directed against the 60-kd peptide. Statistical analyses were performed using Fisher's exact test (two-tailed) with Haldane's adjustment and odds ratio with Cornfield 95% confidence intervals.


RESULTS
Precipitating antibodies against the Ro(SS-A) antigen, determined by gel double-immunodiffusion, were present in an increased frequency in CNS-SS patients with (1) documented clinical CNS disease, (2) focal clinical CNS manifestations and serious complications, (3) large regions of increased signal intensity, consistent with ischemia/infarcts on brain MRI scans or regions of decreased attenuation consistent with infarcts on CT, and (4) abnormal cerebral angiograms consistent with small-vessel angiitis. Finally, the anti-Ro(SS-A) antibody response in CNS was directed against the 60-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 -negative, CNS-SS patients have more serious and extensive CNS disease, some with frank cerebral angiopathy. Anti-Ro(SS-A) antibodies are postulated to play a role in mediating or potentiating vascular injury in CNS-SS.
Alexander
E L
EL
Division of Molecular and Clinical Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224.
Ranzenbach
M R
MR
Kumar
A J
AJ
Kozachuk
W E
WE
Rosenbaum
A E
AE
Patronas
N
N
Harley
J B
JB
Reichlin
M
M
D001323:Autoantibodies / Q000032:analysis*; D001324:Autoantigens / Q000032:analysis*; D001927:Brain Diseases / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D002533:Cerebral Angiography; D006801:Humans; D007249:Inflammation / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology; D008279:Magnetic Resonance Imaging; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins / Q000032:analysis*; D012859:Sjogren's Syndrome / Q000000981:diagnostic imaging / Q000276:immunology* / Q000473:pathology; D014652:Vascular Diseases / Q000000981:diagnostic imaging / Q000276:immunology / Q000473:pathology; D000099207:SS-A Antigen
D001323:Autoantibodies; D001324:Autoantigens; D020733:RNA, Small Cytoplasmic; D012261:Ribonucleoproteins; D000099207:SS-A Antigen; C116006:RO60 protein, human