20. Polyarteritis Nodosa

  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. Eli M. Miloslavsky MD and
  2. John H. Stone MD, MPH

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch20

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Miloslavsky, E. M. and Stone, J. H. (2012) Polyarteritis Nodosa, 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.ch20

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, Allergy and Immunology, Massachusetts General Hospital, Boston, MA, USA

Publication History

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

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Polyarteritis nodosa;
  • fibrinoid necrosis;
  • microaneurysm;
  • vasculitic neuropathy;
  • mononeuritis multiplex;
  • cyclophosphamide;
  • cutaneous PAN;
  • Five Factor Score


Polyarteritis nodosa (PAN) is a systemic vasculitis involving medium and small-sized arteries with findings of fibrinoid necrosis on biopsy. Constitutional symptoms are common and skin lesions as well as mononeuritis multiplex are the most frequent disease manifestations. Renal, gastrointestinal and peripheral motor nerve involvement suggest more severe disease. PAN characteristically spares the lungs and glomerular capillaries. Some patients have a limited cutaneous form of the disease. PAN also occurs in the pediatric population and carries a better prognosis in children than in adults. Diagnosis is made on biopsy of involved tissue. Microaneurysms found on angiography strongly support the diagnosis of PAN. Treatment with glucocorticoids is often sufficient for milder disease, but more severe disease requires the addition of cyclophosphamide. Disease relapse is relatively uncommon and maintenance therapy is generally not recommended.