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Case report: anti-glomerular basement membrane antibody disease with normal renal function
China Nagano1, Yoshimitu Goto2, Katuaki Kasahara3
1Japanese Red Cross Nagoya Daini Hospital, 2-9 Myoken-cho, Showa-ku, Nagoya City, Aichi Prefecture, 466-8650, Japan. chinanagano@nagoya2.jrc.or.jp.
BMC Nephrology
|November 6, 2015
Summary
Anti-glomerular basement membrane (GBM) antibody disease, a rare autoimmune condition, can affect children with normal kidney function. Prompt treatment with plasma exchange, steroids, and cyclophosphamide proved effective in a pediatric case.
Area of Science:
- Nephrology
- Autoimmunology
- Pediatric Nephrology
Background:
- Anti-glomerular basement membrane (GBM) antibody disease is a rare autoimmune disorder.
- It is characterized by rapidly progressive glomerulonephritis due to autoantibodies against the α3-chain of type IV collagen in the GBM.
Observation:
- An 8-year-old girl presented with hematuria and proteinuria detected during a school urine screening.
- Despite normal blood pressure and serum creatinine, persistent proteinuria (urine protein to creatinine ratio ~7 g/g) prompted a renal biopsy.
- Immunofluorescence revealed a linear IgG pattern along the GBM, confirming anti-GBM nephritis with preserved renal function.
Findings:
- The patient received a diagnosis of anti-GBM disease with normal renal function.
- Treatment involved plasma exchange, high-dose intravenous methylprednisolone, and cyclophosphamide.
- This therapeutic regimen rapidly reduced anti-GBM antibody titers and proteinuria.
Implications:
- Anti-GBM disease presenting with normal renal function in children is exceptionally rare.
- Established treatment protocols for pediatric anti-GBM disease are lacking.
- Careful selection of treatment is crucial due to the potential for a poor prognosis in affected children.
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