Patients with ANCA vasculitis treated with therapeutic plasma exchange in General Hospital Zadar, Croatia

Abstract

SUMMARY Introduction Therapeutic plasma exchange (TPE), also known as plasmapheresis, has long been used as a therapy in treating life-threatening conditions in patients with vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCA-associated vasculitis). Materials and methods In this review, we will describe clinical and histopathological characteristics, as well as the outcomes of treatment, of eight patients with ANCA-associated vasculitis, six of whom were treated with TPE. Results Various studies have come to variable conclusions regarding the benefit of plasmapheresis in treating these conditions. For example, the PEXIVAS study did not prove that the use of plasmapheresis with the aim of rapidly reducing ANCA titers had a favorable effect compared to oral glucocorticoids. On the other hand, the results of the MEPEX study showed that plasmapheresis significantly reduced the risk of progression to kidney failure, although in a small sample. In our sample, six out of eight patients underwent plasmapheresis, with five of them also receiving cyclophosphamide, and three receiving a combination of cyclophosphamide + rituximab. Five patients ended up on dialysis, two later underwent kidney transplantation, and two experienced fatal outcomes. Although in a small sample, we did not observe that plasmapheresis, along with additional supportive and pharmacological treatment, resulted in a favorable outcome compared to patients who did not receive it. Conclusion Plasmapheresis remains one of the main treatment options, in line with the ASFA guidelines from 2021 and KDIGO guidelines from 2021, which recommend initiating immunosuppressive induction treatment and plasmapheresis as soon as possible, within 24 hours of diagnosis of rapidly progressive glomerulonephritis (GN), especially with alveolar involvement.