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Scleritis and Wegener's granulomatosis in children
R D Sacks1, E L Stock, S E Crawford
1Department of Ophthalmology, Northwestern University Medical School, Chicago, Illinois 60611.
American Journal of Ophthalmology
|April 15, 1991
Summary
Two pediatric patients with scleritis, a rare eye inflammation, were successfully treated for Wegener's granulomatosis. Early diagnosis and combined therapy of prednisone and cyclophosphamide led to positive outcomes.
Area of Science:
- Ophthalmology
- Rheumatology
- Pediatrics
Background:
- Scleritis, an inflammation of the white outer layer of the eye, can be a manifestation of systemic autoimmune diseases.
- Wegener's granulomatosis (WG), now known as granulomatosis with polyangiitis (GPA), is a rare vasculitis that can affect multiple organs.
Observation:
- Two adolescent female patients presented with scleritis (unilateral and bilateral).
- Clinical features included otitis media, fever, arthralgias, cough, and laboratory findings of elevated erythrocyte sedimentation rate, microscopic hematuria, and positive rheumatoid factor.
- Radiological findings revealed bilateral pulmonary nodules in one patient.
Findings:
- Diagnosis of Wegener's granulomatosis was confirmed via sinus and lung biopsies in both patients.
- Both patients exhibited significant improvement following treatment with a combination of prednisone and cyclophosphamide.
Implications:
- This case series highlights the importance of considering WG/GPA in pediatric patients presenting with scleritis, even with atypical initial symptoms.
- Prompt diagnosis and aggressive immunosuppressive therapy, including corticosteroids and cyclophosphamide, are crucial for managing WG/GPA and preventing severe complications, including ocular involvement.