Granulomatosis with Polyangiitis: A Focus on Differences and Similarities Between Child and Adult Patients.

Abstract

Wegener's granulomatosis (WG), or granulomatosis with polyangiitis (GPA), is a rare autoimmune disease that can cause inflammation in various organs, including the kidneys. Renal involvement in GPA is a major cause of morbidity and mortality in both adults and children, and early detection and effective treatment are essential for preventing renal failure. This review aims to summarize the current evidence on the incidence, clinical features, treatment, and outcomes of renal involvement in children with Wegener's granulomatosis. The incidence of renal involvement in children with GPA ranged from 26% to 56%. Renal involvement is a common and serious complication of GPA in children, and early detection and effective treatment are crucial for preventing renal failure. The most common clinical features were proteinuria, hematuria, and reduced glomerular filtration rate. The majority of children with renal involvement in GPA required treatment with corticosteroids and immunosuppressive agents. The treatment outcomes varied among the studies, with some children achieving remission of renal involvement while others developed end-stage renal disease. Although most features are the same in children and adult patients, this review summed up some important differences between these two different populations. Further studies are needed to identify the most effective treatment strategies for renal involvement in children with GPA.