Scholarlink

Background <p>Double-seropositive anti-glomerular basement membrane (GBM) disease, characterized by the presence of both anti-GBM and anti-neutrophil cytoplasmic autoantibodies (ANCAs), is extremely rare in children. Double-seropositive patients represent a hybrid phenotype combining the severe acute kidney injury of isolated anti-GBM disease with the relapse risk and subsequent need for maintenance treatment of ANCA-associated vasculitis.</p> Clinical presentation <p>Here we present the case of an 11-year-old girl presenting with rapidly progressive glomerulonephritis leading to kidney failure and warranting kidney replacement therapy. Anti-GBM antibodies and myeloperoxidase-ANCAs were both detected in serum. Kidney histopathology revealed over 90% crescents. Despite the aggressive presentation, treatment with high-dose corticosteroids, plasmapheresis, and rituximab led to recovery of kidney function and dialysis discontinuation.</p> Conclusion <p>Our case suggests that rituximab, without cyclophosphamide, may represent a promising therapeutic approach in children with double-seropositive anti-GBM disease, even in severe presentations.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Severe acute kidney failure due to double-seropositive anti-GBM glomerulonephritis in a child: a case for rituximab therapy

  • 期刊论文

  • 发表日期：

    2026年3月5日

查看全文
  • Lien Dossche,
  • Lieselot Peremans,
  • Ann Raes,
  • Amélie Dendooven,
  • Thomas Renson

摘要

Background

Double-seropositive anti-glomerular basement membrane (GBM) disease, characterized by the presence of both anti-GBM and anti-neutrophil cytoplasmic autoantibodies (ANCAs), is extremely rare in children. Double-seropositive patients represent a hybrid phenotype combining the severe acute kidney injury of isolated anti-GBM disease with the relapse risk and subsequent need for maintenance treatment of ANCA-associated vasculitis.

Clinical presentation

Here we present the case of an 11-year-old girl presenting with rapidly progressive glomerulonephritis leading to kidney failure and warranting kidney replacement therapy. Anti-GBM antibodies and myeloperoxidase-ANCAs were both detected in serum. Kidney histopathology revealed over 90% crescents. Despite the aggressive presentation, treatment with high-dose corticosteroids, plasmapheresis, and rituximab led to recovery of kidney function and dialysis discontinuation.

Conclusion

Our case suggests that rituximab, without cyclophosphamide, may represent a promising therapeutic approach in children with double-seropositive anti-GBM disease, even in severe presentations.