{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T17:01:48Z","timestamp":1785862908011,"version":"3.56.0"},"reference-count":24,"publisher":"S. Karger AG","license":[{"start":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T00:00:00Z","timestamp":1781222400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T00:00:00Z","timestamp":1781222400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nephron"],"abstract":"<jats:p>Background: The objective of a multicentre, prospective, observational study of an eculizumab biosimilar (Elizaria\u00ae) was to analyse the efficacy and safety of long-term complement inhibitor therapy in patients with atypical haemolytic uraemic syndrome (aHUS). Materials and Methods: The study included 50 patients aged 1\u201355 years, with 42% of them under the age of 18. The patients received the eculizumab biosimilar in routine clinical practice within 56 weeks; 19 patients (38%) had been treated with eculizumab prior to enrolment, whereas 31 patients (62%) were naive to complement inhibitors. Results: By the end of the study, an increase in platelet count of 39.48 \u00b1 21.7 \u00d7 109\/L and 33.61 \u00b1 24.06 \u00d7 109\/L was reported in treatment-naive and treatment-experienced patients, respectively. By the end of the study, 96% of treatment-experienced patients and 100% of treatment-naive patients had normal platelet counts. The mean LDH activity did not change significantly, with levels of 253.84 \u00b1 122.22 U\/L registered at screening and 245.24 \u00b1 93.52 U\/L at the end of the study. In total, 39 patients (77.6%) at week 21 and 40 patients (80%) at week 52 had no thrombotic microangiopathy (TMA) events. During the study, the proportion of patients with a complete TMA response increased significantly, rising to 8% (p = 0.046). The proportion of patients with an estimated glomerular filtration rate improvement of 15 mL\/min\/1.73 m2 and more at week 52 was 12%. During the safety assessment, 63 adverse events not related to the investigational medicinal product were reported. Conclusion: Long-term complement inhibitor therapy with the eculizumab biosimilar in patients with aHUS has demonstrated a stable effect, a favourable safety profile, and low immunogenicity.<\/jats:p>","DOI":"10.1159\/000552914","type":"journal-article","created":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T16:01:09Z","timestamp":1781280069000},"page":"1-10","source":"Crossref","is-referenced-by-count":0,"title":["Long-Term Therapy with Eculizumab Biosimilar in Patients with Atypical Haemolytic Uraemic Syndrome: Outcomes of a Prospective Observational 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