Pediatric Nephrology

, Volume 14, Issue 3, pp 208–213

Wegener granulomatosis in children and young adults

A case study of ten patients
  • B. G. Stegmayr
  • L. Gothefors
  • B. Malmer
  • D. E. Müller
  • K. Nilsson
  • B. Sundelin
Clinical nephrology / Original Article

DOI: 10.1007/s004670050043

Cite this article as:
Stegmayr, B., Gothefors, L., Malmer, B. et al. Pediatr Nephrol (2000) 14: 208. doi:10.1007/s004670050043

Abstract 

This retrospective study reports seven children and three young adults (aged 11–30 years) who suffered from Wegener granulomatosis. Nine represent consecutive patients admitted to the Division of Nephrology over a period of 23 years. All patients had respiratory tract symptoms and renal involvement on admission. In several patients infiltrates on chest X-ray developed within 2 weeks of onset of symptoms. All patients survived. The median observation period was 9 years (range 13 months to 23 years). One patient progressed to end-stage renal disease. Nine patients initially received cyclophosphamide and steroids. After a median period of 9 months (range 6–31 months) the cyclophosphamide was replaced by azathioprine. Relapses occurred after a median of 28 months (range 4–120 months) in 80% of patients, in six of the eight patients causing a definite decrease in kidney function. We believe that early diagnosis and initiation of therapy reduce the extent of organ damage. Since relapses are frequent, these patients should be evaluated frequently.

Key words Wegener granulomatosis Renal failure Respiratory failure Inflammation Pulmonary infiltrates 

Copyright information

© IPNA - International Pediatric Nephrology Association New York, USA 2000

Authors and Affiliations

  • B. G. Stegmayr
    • 1
  • L. Gothefors
    • 4
  • B. Malmer
    • 4
  • D. E. Müller
    • 2
  • K. Nilsson
    • 5
  • B. Sundelin
    • 3
  1. 1.Department of Internal Medicine, University Hospital, S-90185 Umeå, Sweden e-mail: Bernd.Stegmayr@medicin.umu.se Tel.: +46-90-785-0000, Fax: +46-90-134-550SE
  2. 2.Wiefel University Children’s Hospital, Hamburg, GermanyDE
  3. 3.Department of Pathology, Karolinska Hospital, Stockholm, SwedenSE
  4. 4.Department of Pediatrics, University Hospital, Umeå, SwedenSE
  5. 5.Department of Respiratory Diseases, University Hospital, Umeå, SwedenSE