Teenage Risk Behaviours with Chronic Kidney Disease: A Midwest Paediatric Nephrology Consortium Study

Author(s): Corina Boers*

Objective: To compare and contrast the therapy of paediatric Granulomatosis with Polyangiitis (GPA) between paediatric rheumatologists and nephrologists. Methods: The Midwest Paediatric Nephrology Consortium Group (MWPNC) and a global email listserv for paediatric rheumatology received a voluntary survey in 2016–2017. Three clinical scenarios (A–C) were used to collect data on general practise traits and preferences for induction management: newly diagnosed GPA with glomerulonephritis, GPA with quickly progressing glomerulonephritis, and GPA with pulmonary haemorrhage. Additionally, preferences for GPA maintenance drugs, disease monitoring, and therapy of GPA with end-stage renal illness were identified. Results: The MWPNC membership responded at a rate of 68%, and the number of rheumatologist respondents was equal. According to survey findings, rheumatologists prefer Rituximab with Cyclophosphamide over nephrologists for induction in Scenarios A and B, while nephrologists choose Cyclophosphamide in Scenario A. Although low overall, Plasmapheresis rates increased for Scenarios A, B, and C for both disciplines, regarding the length of either the diagnosis process or maintenance therapy, there was no apparent agreement. Compared to nephrologists, rheumatologists more usually choose Rituximab for maintenance and induction. Additionally, the use of Mycophenolate Mofetil was higher than anticipated for both specialties. Conclusion: This study has identified significant disparities in how rheumatologists and nephrologists treat this condition. It emphasises the necessity of carefully planned clinical trials in paediatric GPA patients and makes clear that both specialities must be represented during efforts to reach consensus and plan clinical studies.