Peripheral inflammatory vascular disease in Sjögren's syndrome. Association with nervous system complications

Abstract

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.

Evaluation of possible factors affecting contrast sensitivity function in patients with primary Sjögren’s syndrome

“…However, it has been reported that pSS patients may also suffer from visual acuity changes or visual quality disturbances as a result of optic neuropathy, which may occur before or at the same time as the diagnosis of pSS (1)(2)(3) . Thus, when evaluating the visual quality of pSS patients, both functional and morphological analyses of the optic nerve should also be performed in addition to examination of the ocular surface structures.…”

Sjögren’s Syndrome: Autoantibodies to Cellular Antigens

“…Furthermore, it is considered a marker of extraglandular involvement since it is associated with lymphadenopathy, splenomegaly and vasculitis [54,55].…”

A case of primary Sjögren’s syndrome presenting primarily with central nervous system vasculitic involvement

“…Alexander and coworkers displayed biopsy-proven peripheral vasculitis accompanying CNS complications frequently and necrotizing spinal arteritis at autopsy in a patient, supporting the vasculitic theory [9]. Other studies have demonstrated that a vasculitic cutaneous involvement was frequent in pSS [10][11][12][13]. Other studies by Alexander and colleagues showed that only 1 of 13 patients had stenosis of the internal carotid artery [10].…”