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Polyarteritis nodosa

John H Stone1

  • 1Division of Rheumatology, Johns Hopkins Vasculitis Center, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA. jstone@jhmi.edu

JAMA
|September 28, 2002
PubMed

Insights

Polyarteritis nodosa (PAN), a vasculitis, can be challenging to diagnose. This case highlights successful treatment with cyclophosphamide after a decade-long misdiagnosis of adult-onset Still disease.

Area of Science:

  • Rheumatology
  • Vasculitis
  • Immunosuppressive therapy

Background:

  • Polyarteritis nodosa (PAN) is a rare systemic vasculitis.
  • Diagnostic challenges in vasculitis can lead to delayed treatment.
  • Historical context of PAN, referencing Kussmaul and Maier's initial description.

Observation:

  • A 30-year-old male patient presented with a 12-year history of daily fevers, tachycardia, and distal extremity ulcers.
  • The patient developed mononeuritis multiplex, a neurological complication.
  • Initial misdiagnosis as adult-onset Still disease due to fever patterns.

Findings:

  • Long-term prednisone therapy was insufficient for disease control.
  • Addition of daily cyclophosphamide led to the first remission in over a decade.
  • Successful withdrawal of all immunosuppressive medications post-remission.

Implications:

  • This case underscores the importance of considering PAN in complex vasculitis presentations.
  • Effective management of refractory PAN may involve cyclophosphamide.
  • Long-term remission and potential drug discontinuation are achievable in PAN.

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