Vasculitis and Sjögren’s Syndrome

  • George E. Fragoulis
  • Haralampos M. Moutsopoulos
  • John H. Stone


Sjögren’s syndrome (SjS) is a chronic autoimmune disorder characterized by dysfunction and destruction of exocrine glands, principally the salivary and lacrimal glands. Vasculitis, one of the major extraglandular manifestations of SjS, frequently heralds the need for systemic therapy and serves as an indicator of a risk for poor outcomes in many patients. Poor outcomes associated with the development of ­vasculitis may include direct complications of the vascular inflammation, such as complications of cutaneous ulceration and vasculitic neuropathy, as well as the side effects of the intensive treatment sometimes required to manage vasculitis. The occurrence of vasculitis along with certain other clinical and serologic features (e.g., salivary gland enlargement and low C4 levels) are markers for patients at increased risk for B cell lymphoma development.


Leukocytoclastic Vasculitis Cutaneous Vasculitis Palpable Purpura Mesangial Proliferative Glomerulonephritis Vasculitic Neuropathy 
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Copyright information

© Springer-Verlag London Limited 2011

Authors and Affiliations

  • George E. Fragoulis
    • 1
  • Haralampos M. Moutsopoulos
    • 1
  • John H. Stone
    • 2
  1. 1.Department of Pathophysiology, School of MedicineNational University of AthensAthensGreece
  2. 2.Division of Rheumatology, Allergy and ImmunologyMassachusetts General HospitalBostonUSA

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