Polyarteritis nodosa

JAMA. 2002 Oct 2;288(13):1632-9. doi: 10.1001/jama.288.13.1632.

Abstract

Polyarteritis nodosa (PAN) is regarded rightly as the grandfather of the vasculitides. In this Grand Rounds, the case of a 30-year-old man with a 12-year illness is described. The patient presented with daily fevers, tachycardia, and cutaneous ulcers on his distal extremities. He eventually developed mononeuritis multiplex. Because of the striking pattern of his fevers, he was diagnosed for many years as having adult-onset Still disease. Following the addition of daily cyclophosphamide to his long-standing regimen of prednisone, the patient's disease entered remission for the first time in more than a decade. He was ultimately able to discontinue all of his immunosuppressive medications. The case is discussed in the context of the first patient ever described with PAN, the classic report of Kussmaul and Maier.

Publication types

  • Case Reports
  • Clinical Conference
  • Historical Article

MeSH terms

  • Adult
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Diagnostic Errors
  • Fever
  • Glucocorticoids / therapeutic use
  • History, 19th Century
  • History, 20th Century
  • History, 21st Century
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Indomethacin / therapeutic use
  • Male
  • Polyarteritis Nodosa* / diagnosis
  • Polyarteritis Nodosa* / epidemiology
  • Polyarteritis Nodosa* / history
  • Polyarteritis Nodosa* / therapy
  • Prednisone / therapeutic use
  • Still's Disease, Adult-Onset / diagnosis
  • Still's Disease, Adult-Onset / drug therapy

Substances

  • Anti-Inflammatory Agents, Non-Steroidal
  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Prednisone
  • Indomethacin