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

Arthritis Rheum. 1985 Dec;28(12):1341-7. doi: 10.1002/art.1780281205.


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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Central Nervous System Diseases / etiology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Nervous System Diseases / drug therapy
  • Nervous System Diseases / etiology*
  • Nervous System Diseases / pathology
  • Neurologic Examination
  • Peripheral Nervous System Diseases / etiology
  • Retrospective Studies
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / pathology
  • Skin Diseases / pathology
  • Vasculitis / complications*
  • Vasculitis / drug therapy
  • Vasculitis / pathology


  • Adrenal Cortex Hormones