Rescue of kidney function in a toddler with anti-GBM nephritis

A Bjerre,J Grotta,K Hogasen,T Tangeraas,C Dorje,H Scott

doi:10.1093/ckj/sfs146

Abstract

Anti-glomerular basement membrane (anti-GBM) nephritis is rare in childhood with few published cases. We report a 19-month-old boy with rapidly progressive glomerulonephritis (RPGN) due to anti-GBM nephritis. Treatment was started under 2 weeks after presentation and included plasma exchange, intravenous high-dose methylprednisolone, intravenous cyclophosphamide and mycophenolate as mainstay medication. The treatment was rapidly effective with immediate decrease in anti-GBM titres and plasma creatinine. Three years after presentation, the boy has normal kidney function, blood pressure and no residual disease. The successful outcome was likely due to the rapid recognition of the anti-GBM antibodies as the cause of RPGN and aggressive primary treatment.

Highlights

Anti-glomerular basement membrane disease is characterized by rapidly progressive glomerulonephritis (RPGN) caused by autoantibodies against the α3-chain of type IV collagen in the GBM [1]
We report a case of a 19-month-old boy with severe disease with RPGN, early recognition and an excellent outcome
Three months after the first admission, his plasma creatinine was 25 μmol/L and albumin 42 g/L; his plasma creatinine. This case demonstrates that rapid diagnosis and prompt aggressive treatment of anti-GBM nephritis can reverse a serious condition with a potentially poor prognosis

Summary

Introduction

Anti-glomerular basement membrane (anti-GBM) disease is characterized by rapidly progressive glomerulonephritis (RPGN) caused by autoantibodies against the α3-chain of type IV collagen in the GBM [1]. We report a case of a 19-month-old boy with severe disease with RPGN, early recognition and an excellent outcome. The enzyme linked immuno assay (ELISA) test for anti-GBM (Orgentec, Alegria) was strongly positive with >200 U/mL The immunofluorescence (IF) test for anti-GBM on primate kidney tissue slides (Inova) was positive with a titre of >40

Results

Conclusion