Combination of Anti-Glomerular Basement Membrane Antibodies & Anti-Neutrophil Cytoplasmic Antibodies: A Curious Presentation in a Child-Case Report

Abstract: Anti-GBM disease is rare in all age groups. Available data estimate the annual incidence of anti-GBM disease as one case per million in the general population, and this disorder is even less common in children. It is characterized by the presence of circulating autoantibodies to the GBM and linear deposition of immunoglobulin G (IgG) along the GBM and alveolar basement membrane. Anti- GBM disease is the most aggressive form of RPGN and may involve the kidneys alone (Goodpasture disease), or be accompanied by pulmonary hemorrhage (Goodpasture syndrome). Circulating antibody level in anti-GBM disease correlates with the severity of renal disease and serum creatinine concentrations. Unless the diagnosis is made early and therapy is instituted ly, anti-GBM disease rapidly progresses to renal failure and end-stage renal disease (ESRD), with a fatal outcome in approximately one-half of all cases. We observed anti-GBM disease in a 13-year-old female child. We present this case of renal failure with anti-GBM and p-ANCA antibodies positive. This patient with dual antibodies is considered to be a vasculitis-variant of anti-GBM antibody nephritis. This patien had atypical presentation, and she presented late to hospital. Recurrence rate is expected to be higher in such patients. We reviewed the literature of cases and studies on crescentic glomerulonephritis with anti-GBM and p-ANCA positive.

Keywords: RPGN, anti GBM antibodies, end stage renal disease

Edition: Volume 7 Issue 9, September 2018,

Pages: 806 - 810