37. Vasculitis and Sjögren's Syndrome

  1. Gary S. Hoffman MD, MS2,
  2. Cornelia M. Weyand MD, PhD3,
  3. Carol A. Langford MD, MHS2 and
  4. Jörg J. Goronzy MD, PhD3
  1. George E. Fragoulis MD and
  2. Haralampos M. Moutsopoulos MD, FACP, FRCP(hc), Master ACR

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch37

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Fragoulis, G. E. and Moutsopoulos, H. M. (2012) Vasculitis and Sjögren's Syndrome, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch37

Editor Information

  1. 2

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 3

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. Department of Pathophysiology, School of Medicine, University of Athens, Athens, Greece

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Vasculitis;
  • Sjögren's syndrome;
  • leukocytoclastic;
  • acute necrotizing;
  • immunocomplexes;
  • cryoglobulins;
  • palpable purpura;
  • peripheral neuropathy;
  • urticarial lesions;
  • B-cell lymphoma


Sjögren's syndrome (SS) is a chronic autoimmune disorder characterized by dysfunction of the exocrine glands. Among its various clinical symptoms, the main of which are keratoconjuctivitis sicca and dry mouth, vasculitis is observed in about 15% of patients. It is usually manifest with skin lesions but rarely can affect vital organs. SS patients with vasculitis have some distinct laboratory characteristics including high titers of rheumatoid factor, high levels of gamma-globulins, low serum complement levels and presence of cryoglobulins, usually type II (monoclonal IgMk with rheumatoid factor activity).

Vasculitis in SS patients has been found to correlate with B-cell lymphoma development and increased mortality. Treatment of vasculitis includes local therapy for limited skin vasculitis, while systemic therapy with corticosteroids and other immunosuppressive agents are used for more severe disease. Anti-CD 20 therapy may also be beneficial.