Successful treatment of childhood onset refractory polyarteritis nodosa with tumor necrosis factor alpha blockade.
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.
Feinstein
Jeffrey
J
Department of Rheumatology, Wilford Hall Medical Center, 2200 Bergquist Drive Ste. 1, Lackland AFB, San Antonio, TX 78236, USA. Jeffrey.Feinstein@lackland.af.mil
Arroyo
Ramon
R
D002675:Child, Preschool; D000068800:Etanercept; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D008297:Male; D010488:Polyarteritis Nodosa / Q000188:drug therapy* / Q000473:pathology; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
D007074:Immunoglobulin G; D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha; D000068800:Etanercept