Two histopathologic types of inflammatory vascular disease (IVD) occur in Sjogren'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.
Peripheral nervous system disease is an establishled complication of primary Sjogren's syndrome (SS) (l), and central nervous system (CNS) disease in SS is being recognized increasingly. In an early study of 75 SS patients, we reported that CNS abnormalities occurred in 11 (15%) (2). Recently, we have described a group of 36 men with primary SS, 9 (25%) of whom had CNS disease (3). It is our growing impression that nervous system complications in SS patients are not uncommon and can constitute a significant cause of From the