Abstract
The authors reviewed the medical histories and radiological examinations of five pediatric patients with a histologic diagnosis of Wegener's granulomatosis (WG) seen over a six year period in whom a total of 22 thoracic CT scans were performed. Involvement of both the upper and lower respiratory tracts was seen in all patients at presentation. One patient had subglottic stenosis necessitating tracheotomy. Pulmonary hemorrhage occurred in three patients at initial diagnosis. Classic cavitary lung nodules were seen in two patients — one at initial presentation, the other at relapse. The plain radiographic lower respiratory tract manifestations of pediatric WG were protean both at initial presentation and during follow up. Similarly, disease expression was highly variable on thoracic CT examinations but, overall, multifocal parenchymal infiltrates with or without small peripheral nodules were the commonest thoracic CT manifestations. As a consequence of cytotoxic and corticosteroid therapy the long-term prognosis of WG has improved considerably in recent years. Knowledge of the varied patterns of the primary disease and potential for iatrogenic complications are necessary for successful radiologic assessment of pediatric patients with WG.
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Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern 98: 76
Horn RG, Fauci AS, Rosenthal AS, Wolff SM (1974) Renal biopsy pathology in Wegener's granulomatosis. Am J Pathol 74: 423
Singer J, Suchet I, Horwitz T (1990) Paediatric Wegener's granulomatosis: two case histories and a review of the literature. Clin Radiol 42: 50
Hall SL, Miller LC, Duggan E, Mauer SM, Beatty EC, Hellerstein S (1985) Wegener granulomatosis in pediatric patients. J Pediatr 106: 739
Canny GJ, Laxer RM, Levison H (1988) Recurrent otitis media, sinusitis and pulmonary infiltrates in a 13-year-old girl. Ann Allergy 60: 201
Leavitt RY, Fauci AS, Bloch DA et al. (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 33: 1101
Aberle DR, Gamsu G, Lynch D (1990) Thoracic manifestations of Wegener granulomatosis: diagnosis and course. Radiology 174: 703
Neumann G, Benz-Bohm G, Rister M (1984) Wegener's granulomatosis in childhood. Review of the literature and case report. Pediatr Radiol 14: 267
Cohen MR, Gore RM, August CZ, Ossoff RH (1984) Tracheal and bronchial stenosis associated with mediastinal adenopathy in Wegener granulomatosis: CT findings. J Comput Assist Tomogr 8: 327
Paling MR, Roberts RL, Fauci AS (1982) Paranasal Sinus Obliteration in Wegener Granulomatosis. Radiology 144: 539
Bohlman ME, Ensor RE, Goldman SM (1984) Primary Wegener's granulomatosis of the trachea: radiologic manifestations. South Med J 77: 1318
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McHugh, K., Manson, D., Eberhard, B.A. et al. Wegener's granulomatosis in childhood. Pediatr Radiol 21, 552–555 (1991). https://doi.org/10.1007/BF02012595
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DOI: https://doi.org/10.1007/BF02012595