Severe systemic lupus erythematosus with thrombotic microangiopathy: rescue therapy with pegcetacoplan.
Christian Stirnkorb, Verena Ney, Bhavna Issar et al.
✦ AI-curated · Sources linked
Case in brief
A 12-year-old girl with severe lupus nephritis and refractory thrombotic microangiopathy remained dialysis-dependent for several months despite intensive immunosuppression, including terminal complement blockade with eculizumab. After treatment was switched to pegcetacoplan, haemolytic activity fell and kidney function steadily improved. Following more than 8 months of haemodialysis, she was able to discontinue dialysis.
What made this case unusual
The thrombotic microangiopathy persisted despite eculizumab and intensive immunosuppression, with improvement reported after switching to pegcetacoplan.
Diagnostic / clinical pearl
In this case of lupus-associated thrombotic microangiopathy, persistent haemolysis and kidney dysfunction despite eculizumab were followed by improvement after a switch to pegcetacoplan.
Why it matters
The case suggests pegcetacoplan may have therapeutic potential in lupus-associated thrombotic microangiopathy, although a single report cannot establish efficacy.