Peripheral inflammatory vascular disease in Sjögren's syndrome. Association with nervous system complications.
Two histopathologic types of inflammatory vascular disease (IVD) occur in Sjögren's syndrome (SS): mononuclear IVD (MIVD) and neutrophilic IVD (NIVD). We describe 50 SS patients with IVD (30 with NIVD and 20 with MIVD). Thirty-three (66%) of the SS patients with biopsy-documented IVD had nervous system disease unattributable to other causes. Nineteen patients (58%) had involvement of both the central and peripheral nervous systems, while 9 had peripheral and 5 had central nervous system dysfunction alone. Patients with both histopathologic types of IVD were at risk for the development of nervous system abnormalities (57% of NIVD patients and 80% of MIVD patients). Indirect evidence is presented which suggests that IVD may play a role in the immunopathogenesis of nervous system disease, at least in a subset of SS patients.
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D002493:Central Nervous System Diseases / Q000209:etiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009422:Nervous System Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D009460:Neurologic Examination; D010523:Peripheral Nervous System Diseases / Q000209:etiology; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000150:complications* / Q000473:pathology; D012871:Skin Diseases / Q000473:pathology; D014657:Vasculitis / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D000305:Adrenal Cortex Hormones