40. Vasculitis and Relapsing Polychondritis

  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. Tanaz A. Kermani MD and
  2. Kenneth J. Warrington MD

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch40

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Kermani, T. A. and Warrington, K. J. (2012) Vasculitis and Relapsing Polychondritis, 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.ch40

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. Division of Rheumatology, College of Medicine, Mayo Clinic, Rochester, MN, USA

Publication History

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

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244

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Keywords:

  • Relapsing polychondritis;
  • chondritis;
  • vasculitis;
  • aortitis;
  • aortic aneurysm;
  • corticosteroids

Summary

Relapsing polychondritis (RPC) is an autoimmune disorder characterized by multisystem involvement of cartilaginous structures. The pathogenesis of this disease is not well understood. Vasculitis in RPC may involve large, medium or small-sized arteries. Aortic disease in RPC may result in aneurysm formation or dissection. Other vasculitic manifestations including scleritis, episcleritis, retinal vasculitis, cutaneous vasculitis, coronary vasculitis and cerebral vasculitis have all been reported in association with RPC. In up to 10% cases, RPC may be associated with systemic vasculitis, mainly granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis (MPA). Corticosteroids and immunosuppressive medications remain the mainstay of therapy. RPC patients with an associated systemic vasculitis have a poorer prognosis.