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Wegener's granulomatosis in the pediatric age group
Insights
Pediatric Wegener's granulomatosis (WG) is rare, often starting in the second decade. Early diagnosis and a combination therapy of nitrogen mustard, adrenocorticotropic hormone or prednisone, and cyclophosphamide improve survival rates.
Area of Science:
- Rheumatology
- Pediatric Nephrology
- Pulmonology
Background:
- Wegener's granulomatosis (WG) is a rare systemic vasculitis affecting young patients.
- The disease presents with a triad of sinonasal, pulmonary, and renal involvement.
- Historically, WG had a poor prognosis, with high mortality rates.
Purpose of the Study:
- To describe six new cases of Wegener's granulomatosis in patients under 21 years old.
- To review the clinical presentation, diagnostic findings, and treatment outcomes for pediatric WG.
- To highlight the improved survival rates with modern therapeutic regimens.
Main Methods:
- Case series of six pediatric patients with Wegener's granulomatosis.
- Review of 11 previously reported pediatric cases.
- Analysis of clinical presentation, diagnostic findings, and treatment responses.
Main Results:
- Six new cases add to the limited literature on pediatric WG, with onset in the second decade of life.
- Common symptoms include malaise, fever, sinusitis, epistaxis, and hematuria.
- Pulmonary infiltrates, sinus disease, and renal involvement are typical initial findings.
- Modern treatment regimens have increased survival rates to over 50%.
Conclusions:
- Wegener's granulomatosis in children is rare but presents similarly to adult cases.
- Prompt diagnosis and aggressive treatment are crucial for improving outcomes.
- A recommended regimen involving nitrogen mustard, corticosteroids, and cyclophosphamide shows promise for inducing remission.
Abstract:
Six cases of Wegener's granulomatosis (WG) occurring in patients younger than 21 years are described. Only 11 other cases in the pediatric age group have been reported, and all 17 of these patients had the onset of the disease during the second decade of life. Wegener's granulomatosis is a systemic disease characterized by a clinical triad of paranasal sinus and nasal mucosa involvement, pulmonary infiltration and cavitation, and renal disease with hematuria. The most common presenting symptoms are malaise and fever, sinusitis, epistaxis, and hematuria. Most patients have roentgenographic evidence of pulmonary and sinus disease and laboratory evidence of renal involvement on initial evaluation. The prognosis of WG was formerly dismal; more than 90% of patients died in less than two years, but with recent therapeutic regimens, more than 50% of these patients are surviving. The treatment we recommend consists of nitrogen mustard with adrenocorticotropic hormone or prednisone for the induction of remission, followed by cyclophosphamide and prednisone as maintenance drugs. This regimen has proved effective in inducing a remission in four of four patients.