Prikaz bolesnika s ANCA-vaskulitisom liječenih terapijskom izmjenom plazme u Općoj bolnici Zadar u Republici Hrvatskoj

Dragan Klarić, Matija Horaček, Marta Klarić, Borna Cvečić

Uvod: Terapijska izmjena plazme (TPE, prema engl. therapeutic plasma exchange) ili plazmafereza je dugo primjenjivana terapija u liječenju po život opasnih stanja bolesnika s vaskulitisom povezanim s antineutrofilnim citoplazmatskim protutijelima (ANCA, prema engl. anti-neutrophil cytoplasmic antibody; AAV, prema engl. ANCA-associated vasculitis). Materijali i metode: U ovom prikazu osam bolesnika s AAV-om, od kojih je šest liječenih s TPE-om, opisat ćemo njihove kliničke i histopatološke karakteristike te ishode njihova liječenja. Rezultati: Različite studije došle su do varijabilnih zaključaka o koristi plazmafereze u liječenju opisanih stanja. Na primjer, studija PEXIVAS nije dokazala da primjena plazmafereze s ciljem brzog smanjenja titra ANCA ima povoljniji učinak u odnosu na oralne glukokortikoide. S druge strane, rezultati studije MEPEX pokazali su da je plazmafereza značajno smanjila rizik od progresije u zatajenje bubrega, iako na malom uzorku. U našem uzorku, šest od osam pacijenata podvrgnuto je plazmaferezi, a uz to je njih petero primalo ciklofosfamid, a troje kombinaciju ciklofosfamida i rituksimaba. Petero bolesnika završilo je na dijalizi, dvoje kasnije i na transplantaciji bubrega, a dvoje je doživjelo smrtni ishod. Iako na malom uzorku, nismo primijetili da je plazmafereza, uz dodatno nadomjesno i farmakološko liječenje, imala povoljniji ishod u odnosu na pacijente kod kojih nije primijenjena. Zaključak: Plazmafereza je i dalje jedna od glavnih opcija liječenja, što je u skladu sa smjernicama ASFA iz 2021. godine i smjernicama KDIGO iz 2021. godine prema kojima imunosupresivno indukcijsko liječenje i plazmaferezu treba započeti što je prije moguće (u roku od 24 sata) od postavljanja dijagnoze brzonapredujućeg glomerulonefritisa (GN), pogotovo sa zahvaćenošću plućne bazalne membrane i s alveolarnim krvarenjem.

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 ANCAassociated 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.