Multimodal Management of Anti-GBM Disease Complicated by Secondary Complement-Mediated Thrombotic Microangiopathy in a Patient Intolerant to Plasma Exchange: A Case Report.
Xiangyu Yang, Ping Guo, Lisha Ma et al.
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Case in brief
A 46-year-old woman with anti-GBM disease developed thrombotic microangiopathy, with preserved ADAMTS13 activity (72.04%) and 2.3% schistocytes. Soluble C5b-9 was elevated during severe concurrent infection, suggesting complement activation in a secondary setting. Therapeutic plasma exchange was stopped because of oxygen desaturation, fever, and dizziness. She received seven sessions of protein A immunoadsorption, during which anti-GBM antibody levels fell from > 400 to 20.80 RU/mL, and eculizumab on Days 7, 14, and 30 for persistent complement activation. Hematologic and biochemical parameters stabilized and gradually improved during combined therapy.
What made this case unusual
The case combined anti-GBM disease with TMA and ongoing complement activation during severe infection, while intolerance prevented use of standard plasma exchange. Protein A immunoadsorption was used for antibody removal alongside short-term eculizumab.
Diagnostic / clinical pearl
In anti-GBM disease with TMA, preserved ADAMTS13 activity and elevated soluble C5b-9 during severe infection may support consideration of secondary complement-mediated TMA rather than assuming primary aHUS; a single case cannot establish causality.
Why it matters
When plasma exchange is not tolerated, protein A immunoadsorption may be a feasible alternative for antibody removal; the report also describes short-term complement inhibition in selected cases with persistent activation.