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%). 1. Introduction Wegener’s granulomatosis (WG) is a chronic systemic vasculitis mainly affecting the upper and lower respiratory tracts and kidneys that usually involve small to medium-sized arteries [1]. This condition that also known as granulomatosis polyangiitis (GPA) was described by McBride in 1897 as a midfacial granuloma syndrome, but the complete picture was described in 1930s [2, 3]. This vasculitis was characterized by the formation of necrotising granuloma in the respiratory tracts and necrotizing pauci-immune glomerulonephritis. The triad of upper and lower respiratory tract inflammation and renal disease are characteristic for GPA. The etiology is unknown, but it is usually associated with antineutrophil cytoplasmic antibody (ANCA) that was detected in predominantly cytoplasmic form (cANCA). The peak incidence of GPA is in the fourth to sixth decades [4–6] and it is rare in children, so both the American college of Rheumatology (ACR) classification criteria [7] and the subsequent Chapel Hill consensus conference (CHCC) disease definitions [8] are based largely on adult data. Recently, a consensus committee of pediatric rheumatology and nephrology experts, under the auspices of the European League Against Rheumatism (EULAR) and the Pediatric Rheumatology European Society (PReS), proposed a system of classification for vasculitis that took into account existing pediatric knowledge and experience [9]. At onset, nonspecific complaints of fever, malaise, fatigue, and weight loss are very common. A large majority of children with GPA present with multiple organ involvement. There is no comprehensive study on GPA in Iran. So, the aim of this study was to describe pediatric patients with GPA from all pediatrics rheumatology
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