WEGENER GRANULOMATOSIS
Wegener granulomatosis (Granulomatosis with polyangiitis or GPA) is a rare small-vessel vasculitis in children, affecting respiratory and renal vasculature, with characteristic necrotizing granuloma formation.
Clinically, most cases present in or beyond adolescence, as chronic sinusitis, chronic pulmonary disease with hemoptysis or as glomerulonephritis.
Diagnosis rests on chest X-ray/CT-scan to reveal nodular opacities and necrotising granulomatous angiitis on renal/lung/sinus biopsy. ANCA (antineutrophilic cytoplasmic antibodies) are positive in gt;90% cases and the presence of anti-PR3 is specific.
Treatment includes immunosuppressive therapy with steroids, cyclophosphamide or methotrexate, though.
Complications d ue to enlarging granulomas, e.g. orbital or ear involvement, pulmonary hemorrhage and chronic renal failure are common.
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