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ISRN Rheumatology
Volume 2013 (2013), Article ID 694928, 4 pages
http://dx.doi.org/10.1155/2013/694928
Research Article

Evaluation of 10-Year Experience of Wegener’s Granulomatosis in Iranian Children

1Pediatric Rheumatology Research Group, Rheumatology Research Center, Tehran University of Medical Sciences, Tehran, Iran
2Growth and Development Research Center, Tehran University of Medical Sciences, Tehran, Iran
3Department of Pediatrics, Tehran University of Medical Sciences, Tehran, Iran
4Department of Pediatrics, Shahid Beheshti University of Medical Sciences, Tehran, Iran
5Department of Pediatrics, Ardabil University of Medical Sciences, Ardabil, Iran
6Division of Pediatric Rheumatology, Children’s Medical Center, No. 62 Dr. Gharib Sreet, Keshavarz Boulevard, Tehran 14194, Iran

Received 31 May 2013; Accepted 3 July 2013

Academic Editors: W. H. Abdulahad and P. A. Gatenby

Copyright © 2013 Fatemeh Tahghighi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Wegener’s granulomatosis or granulomatosis polyangiitis (GPA) is an uncommon chronic systemic vasculitis in children. The aim of this study was to describe pediatric patients with GPA in Iran. We studied records of all patients with GPA diagnosis who were referred to all Iranian Pediatric Rheumatology Division from 2002 to 2011. A total of 11 patients (5 females and 6 males) enrolled in this study. In children less than 15 years old, the prevalence of GPA is 0.6 per million. The mean age of GPA diagnosis was 11 years and average delay diagnosis was 20 months. Mortality rate was 18.1% due to pulmonary vasculitis and infection. The most common organ system involvement was upper and lower respiratory tract involvement (81.8% and 63.9%, resp.). Other common manifestations were renal (36.3), skin (27.2%), and eye involvement (18.2%).