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 Vol. 13 No. 4, April 2005
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AAP Grand Rounds 13:45 (2005)
© 2005 American Academy of Pediatrics

RHEUMATOLOGY

Therapy for Wegener’s Granulomatosis

Source: Stone JH and The Wegener’s Granulomatosis Etanercept Trial Research Group. Etanercept plus standard therapy for Wegener’s granulomatosis. New Engl J Med. 2005;352:351–361.[Abstract/Free Full Text]

The first 20% of the full text of this article appears below.

The majority of patients diagnosed with Wegener’s granulomatosis (WG), a multi-organ, inflammatory disease that most often affects the kidneys and respiratory tract, will go into remission with the standard treatment of glucocorticoids and cyclophosphamide. However, over half of these patients will eventually have a disease flare, necessitating reintroduction of therapy. Side effects from these medications cause significant morbidity and mortality.1,2 These researchers from the Johns Hopkins Vasculitis Center conducted a multicenter, randomized, double-blind, placebo-controlled trial of etanercept, a soluble tumor necrosis factor {alpha} (TNF-{alpha}) inhibitor, for the maintenance of remission in WG.

The choice of etanercept was based on several factors: (1) in animal models, granuloma formation is markedly decreased by the inhibition of TNF-{alpha}; (2) elevated levels of TNF-{alpha} are produced by CD4+ cells of patients with WG; (3) TNF-{alpha} receptor serum levels correlate with disease activity in patients with WG; (4) TNF-{alpha} positive cells infiltrate renal lesions; (5) the ability of antineutrophil cytoplasmic antibodies (ANCA) to stimulate the degranulation of neutrophils is markedly enhanced in vitro by priming the activated neutrophils with TNF-{alpha}; (6) disease-activity scores were markedly reduced in patients with WG . . . [Full Text of this Article]

Susan Ballinger, MD, FAAP1 and Bernhard Wiedermann, MD, FAAP2
1 Pediatric Rheumatology, Indiana University School of Medicine, Indianapolis, IN
2 Pediatric Infectious Disease, Children’s National Medical Center, Washington, DC