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Whole-Kidney Three-Dimensional Staining with CUBIC

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Published on: July 18, 2022

Three kidneys, two diseases, one antibody?

Nolwenn Lorcy¹, Nathalie Rioux-Leclercq, Marie-Laure Lombard

¹Service de Néphrologie, CHU Pontchaillou, Rennes, France.

Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association

|August 5, 2011

Summary

Anti-factor H antibody causes kidney diseases like membranoproliferative glomerulonephritis (MPGN) and thrombotic microangiopathy (TMA). This case highlights MPGN and TMA

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Area of Science:

Nephrology
Immunology
Complement System

Background:

Membranoproliferative glomerulonephritis (MPGN) and thrombotic microangiopathies (TMA) are serious kidney diseases.
Anti-factor H antibodies have recently been implicated in the pathogenesis of these conditions.

Observation:

A patient with anti-factor H antibody developed MPGN in her native kidney.
The MPGN rapidly recurred in her first kidney transplant.
Thrombotic microangiopathy (TMA) developed in her second kidney transplant, despite treatment.

Findings:

This case demonstrates the link between MPGN and TMA.
Recurrence of MPGN and development of TMA occurred sequentially in kidney grafts.
Treatment with immunosuppression and plasma exchange was insufficient to prevent recurrence or TMA.

Implications:

MPGN and TMA may share common underlying pathogenic mechanisms involving the complement system.
Complete evaluation of the complement pathway, including factor H activity and autoantibodies, is crucial for managing MPGN.
Further research is needed to understand the role of anti-factor H antibodies in kidney diseases and to develop targeted therapies.