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J Clin Rheumatol. 2005 Aug;11(4):219-22.

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

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Department of Rheumatology, Wilford Hall Medical Center, 2200 Bergquist Drive Ste. 1, Lackland AFB, San Antonio, TX 78236, USA.


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.

[Indexed for MEDLINE]

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