35. Rheumatoid Vasculitis

  1. Gary S. Hoffman MD, MS3,
  2. Cornelia M. Weyand MD, PhD4,
  3. Carol A. Langford MD, MHS3 and
  4. Jörg J. Goronzy MD, PhD4
  1. Kimberly P. Liang MD1,
  2. Carl Turesson MD, PhD2 and
  3. Larry W. Moreland MD1

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch35

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Liang, K. P., Turesson, C. and Moreland, L. W. (2012) Rheumatoid Vasculitis, 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.ch35

Editor Information

  1. 3

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

  2. 4

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

Author Information

  1. 1

    Department of Medicine and Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, USA

  2. 2

    Department of Rheumatology, Skåne University Hospital, Lund University, Malmö, Sweden

Publication History

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

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Vasculitis;
  • rheumatoid arthritis;
  • rheumatoid vasculitis;
  • digital ulcers;
  • mononeuritis multiplex;
  • keratitis;
  • coronary arteritis;
  • glomerulonephritis;
  • rheumatoid aortitis;
  • drug-induced vasculitis


Rheumatoid vasculitis (RV) is a rare extra-articular complication of rheumatoid arthritis (RA). It is an inflammatory disorder of small, medium-sized and, least often, large blood vessels. It may affect various organ systems, including the skin, peripheral nerves, central nervous system (CNS), eye, heart, kidneys, lungs and gastrointestinal tract. The most common clinical presentations include leg ulcers and digital ulcers and gangrene; mononeuritis multiplex; scleritis and keratitis; and systemic constitutional symptoms. RV has historically been associated with high rates of morbidity and mortality, and typically occurs in RA patients with high levels of rheumatoid factor (RF) and/or anticyclic citrullinated peptide antibody (CCP), rheumatoid nodules and/or other extra-articular disease manifestations of RA (ExRA). A definitive diagnosis usually is made by obtaining a biopsy of an affected organ and excluding other potential causes of vascular injury (e.g. infection, coagulopathy). Treatment includes high dose glucocorticosteroids and cytotoxic agents.