A 10 year-old-girl with coexistence of anti-glomerular basement membrane antibody nephritis and myeloperoxidase anti-neutrophil cytoplasmic antibodies was able to maintain renal function by plasma exchange therapy and immunosuppressive therapy

Abstract

We report a case of a 10-year-old girl with coexistence of anti-glomerular basement membrane antibody nephritis and myeloperoxidase anti-neutrophil cytoplasmic antibody (ANCA). She initially complained of vomiting and loss of appetite. Serum creatinine (sCr) was at 2.80 mg/dl at the admission, and was elevated to 6.64 mg/dl 12 days later. Kidney biopsy revealed 90% sclerotic lesions and 67% fibrocellular crescentic glomeruli, as well as linear IgG depositions along the glomerular basement membrane (GBM). We diagnosed the patient as rapid progressive glomerular nephritis(RPGN) with anti-GBM glomerulonephritis. We opted for aggressive treatment because of the high percentage of cellular crescents. She received eight rounds of plasma exchange (PE) and six pulses of methylprednisolone (15 mg/kg) therapy, followed by prednisolone (1 mg/kg/day) and cyclophosphamide (2 mg/kg/day). Renal function slightly improved (sCr 2.0 mg/dl); however, it deteriorated after Pneumocystis jirovecci infection. Peritoneal dialysis could be avoided for four months. Her residual renal function was maintained for over 12 months with continuous ambulatory peritoneal dialysis performed twice daily. We should consider aggressive treatment with PE therapy, steroid pulse therapy and immunosuppressive therapy for anti-glomerular basement membrane antibody nephritis. Additionally, the possibility of infectious diseases should be noted.