A Case of Wegener's Granulomatosis with Multi-organ Involvement in Childhood

Lee, Hyun-Kyung; Cho, Hee-Yeon; Cheong, Hae-Il; Choi, Yong; Ha, Il-Soo

doi:2007.11.1.118

J Korean Soc Pediatr Nephrol > Volume 11(1); 2007 > Article

J Korean Soc Pediatr Nephrol 2007;11(1): 118-125. doi: https://doi.org/10.3339/jkspn.2007.11.1.118

다장기 침범을 보인 소아 Wegener 육아종증 1례

이현경, 조희연, 정해일, 최용, 하일수

¹서울대학교 의과대학 소아과학교실
²서울대학교 의과대학 소아과학교실
³서울대학교 의과대학 소아과학교실
⁴서울대학교 의과대학 소아과학교실
⁵서울대학교 의과대학 소아과학교실

A Case of Wegener's Granulomatosis with Multi-organ Involvement in Childhood

Hyun-Kyung Lee, Hee-Yeon Cho, Hae-Il Cheong, Yong Choi, Il-Soo Ha

¹Department of Pediatrics, Seoul National University College of Medicine
²Department of Pediatrics, Seoul National University College of Medicine
³Department of Pediatrics, Seoul National University College of Medicine
⁴Department of Pediatrics, Seoul National University College of Medicine
⁵Department of Pediatrics, Seoul National University College of Medicine

ABSTRACT

Wegener's granulomatosis(WG) is a necrotizing granulomatous small vessel vasculitis with a clinical predilection for involvement of the upper airways, lungs and kidneys. The disease usually manifests in adults between 25 and 50 years of age, but it can also rarely occur-in childhood with some features different from those of adults. WG may be easily overlooked in young patients by misinterpretion of the symptoms as caused by an infectious disease of the respiratory tract. Delayed diagnosis and treatment of the disease may cause more rapid progression of the glomerulonephritis to end stage renal disease. We report a boy who was diagnosed with WG with involvement of multiple organs at 13 years of age.

Key words: Childhood | Wegener's granulomatosis | Multi-organ involvement