Anti-Gbm Disease in Children: Outcomes and Association with Systemic Vasculitis

Abstract

Anti-Glomerular Basement Membrane (anti-GBM) disease is a rare autoimmune disorder affecting the glomerular and alveolar basement membranes. Diagnosis is based on the detection of anti-GBM autoantibodies, along with renal or lung biopsy. Some patients are both anti-GBM and ANCA positive, reflecting an association with systemic vasculitis that has been reported only in some adult cases. Dual positivity of anti-GBM and ANCA is associated with poorer prognosis and higher relapse rates therefore more aggressive and longer treatment is essential. In this case, series we report four cases of children diagnosed with anti-GBM disease that we also screened for signs of systemic vasculitis.