Successful treatment of childhood onset refractory polyarteritis nodosa with tumor necrosis factor alpha blockade

J Clin Rheumatol. 2005 Aug;11(4):219-22. doi: 10.1097/01.rhu.0000173225.41933.83.


Polyarteritis nodosa is a rare systemic necrotizing vasculitis of small- and medium-sized arteries that affects patients of all ages. Its incidence ranges from 2 to 9 per million people. The 5-year survival rate is 13% in untreated patients and 77.6% with modern therapy. Standard treatment includes corticosteroids and cyclophosphamide. Despite aggressive medical management, 22.4% of patients die within 5 years, and of the survivors, medication-induced morbidity is frequent. There is great need for better treatment modalities in terms of safety and efficacy. We report the case of a 5-year-old boy with polyarteritis nodosa refractory to all known standard treatments. After 9 years of persistently active disease, at the age of 14, he was successfully managed with the tumor necrosis factor alpha antagonist, etanercept.

Publication types

  • Case Reports

MeSH terms

  • Child, Preschool
  • Etanercept
  • Humans
  • Immunoglobulin G / therapeutic use*
  • Male
  • Polyarteritis Nodosa / drug therapy*
  • Polyarteritis Nodosa / pathology
  • Receptors, Tumor Necrosis Factor / therapeutic use*
  • Treatment Outcome
  • Tumor Necrosis Factor-alpha / antagonists & inhibitors*


  • Immunoglobulin G
  • Receptors, Tumor Necrosis Factor
  • Tumor Necrosis Factor-alpha
  • Etanercept