Page 16 / Case
Wegener's Granulomatosis is a disease involving Granulomatous inflammation and necrotizing vasculitis of the upper and lower airways and kidneys. There are no validated diagnostic criteria, and there are no case definition criteria validated for pediatrics. Diagnosis is based on the clinical pattern, supportive investigations such as c-ANCA/anti-PR3 and pulmonary imaging, and biopsy of the respiratory tract and kidneys. Certain features of WG are unique to pediatric disease: subglottic, tracheal and endobronchial stenosis; renal involvement correlates with age; and lower treatment-related morbidity. There are no clear guidelines to direct treatment, though the mainstay is immunosuppressive therapy with a combination of high-dose steroids and cyclophosphamide. Methotrexate and azathioprine have been used as initial therapy for limited disease, and for maintenance of remission. Future directions for research into pediatric Wegener's Granulomatosis involve the validation of pediatric case definition criteria, the development of diagnostic criteria, and the elucidation of more specific etiologic factors in order to better direct diagnosis and treatment.
References /
SUMMARY:
Wegener's Granulomatosis is a disease involving Granulomatous inflammation and necrotizing vasculitis of the upper and lower airways and kidneys. There are no validated diagnostic criteria, and there are no case definition criteria validated for pediatrics. Diagnosis is based on the clinical pattern, supportive investigations such as c-ANCA/anti-PR3 and pulmonary imaging, and biopsy of the respiratory tract and kidneys. Certain features of WG are unique to pediatric disease: subglottic, tracheal and endobronchial stenosis; renal involvement correlates with age; and lower treatment-related morbidity. There are no clear guidelines to direct treatment, though the mainstay is immunosuppressive therapy with a combination of high-dose steroids and cyclophosphamide. Methotrexate and azathioprine have been used as initial therapy for limited disease, and for maintenance of remission. Future directions for research into pediatric Wegener's Granulomatosis involve the validation of pediatric case definition criteria, the development of diagnostic criteria, and the elucidation of more specific etiologic factors in order to better direct diagnosis and treatment.
References /