Abstract
Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Fauci, A. S., Wolff, S. M., Johnson, J. S.: Effect of cyclophosphamide upon the immune response in Wegener's granulomatosis. N. Engl. J. Med.285, 1493–1496 (1971)
Fauci, A. S., Wolff, S. M.: Wegener's granulomatosis: studies in eighteen patients and review of the literature. Medicine52: 535–561 (1973)
Godmann, G. C., Churg, H.: Wegener's granulomatosis: pathology and review of the literature. Arch. Path.58, 533–553 (1954)
Israel, H. L., Patchefshy, A. S., Saldana, M. J.: Wegener's granulomatosis, lymphomatoid granulomatosis and benign lymphatic angiitis and granulomatosis of lung. Ann. Int. Med.87, 691–699 (1977)
Liebow, A. A.: Pulmonary angiitis and granulomatosis. Am. Rev. Resp. Dis.108, 1–18 (1973)
Moorthy, A. V., Chesney, R. W., Segar, W. R., Groshong, T.: Wegener granulomatosis in childhood: prolonged survival following cytotoxic therapy. J. Pediat.91, 616–618 (1977)
Norton, W. L., Suky, W., Strunk, S.: Combined corticosteroids and Azathioprine therapy. Arch. Int. Med.121, 554–560 (1968)
Novack, S. N., Pearson, L. M.: Cyclophosphamide therapy in Wegener's grnulomatosis. N. Engl. J. Med.284, 938–942 (1971)
Saldana, M. J., Patchefsky, A. S., Israel, H. L., Atkinson, G. W.: Pulmonary angiitis and granulomatosis. Human Path.8, 391–409 (1977)
Schillitoe, E. S., Lehner, T.: Immunological features of Wegener's granulomatosis. Lancet1974 II, 281–284
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Nespoli, L., Duse, M., Vitiello, M.A. et al. A rapid unfavorable outcome of wegener's granulomatosis in early childhood. Eur J Pediatr 131, 277–282 (1979). https://doi.org/10.1007/BF00444349
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00444349