{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T03:20:20Z","timestamp":1790911220619,"version":"4.1.0"},"reference-count":32,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2021,1,24]],"date-time":"2021-01-24T00:00:00Z","timestamp":1611446400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100006396","name":"Alexion Pharmaceuticals, Inc","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100006396","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,8,30]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Eculizumab modifies the course of disease in patients with atypical haemolytic uraemic syndrome (aHUS), but data evaluating whether eculizumab discontinuation is safe are limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients enrolled in the Global aHUS Registry who received \u22651\u2009month of eculizumab before discontinuing, demonstrated haematologic or renal response prior to discontinuation and had \u22656\u2009months of follow-up were analysed. The primary endpoint was the proportion of patients suffering from thrombotic microangiopathy (TMA) recurrence after eculizumab discontinuation. Additional endpoints included: estimated glomerular filtration rate changes following eculizumab discontinuation to last available follow-up; number of TMA recurrences; time to TMA recurrence; proportion of patients restarting eculizumab; and changes in renal function.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>We analysed 151 patients with clinically diagnosed aHUS who had evidence of haematologic or renal response to eculizumab, before discontinuing. Thirty-three (22%) experienced a TMA recurrence. Univariate analysis revealed that patients with an increased risk of TMA recurrence after discontinuing eculizumab were those with a history of extrarenal manifestations prior to initiating eculizumab, pathogenic variants or a family history of aHUS. Multivariate analysis showed an increased risk of TMA recurrence in patients with pathogenic variants and a family history of aHUS. Twelve (8%) patients progressed to end-stage renal disease after eculizumab discontinuation; seven (5%) patients eventually received a kidney transplant. Forty (27%) patients experienced an extrarenal manifestation of aHUS after eculizumab discontinuation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Eculizumab discontinuation in patients with aHUS is not without risk, potentially leading to TMA recurrence and renal failure. A thorough assessment of risk factors prior to the decision to discontinue eculizumab is essential.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ckj\/sfab005","type":"journal-article","created":{"date-parts":[[2021,1,14]],"date-time":"2021-01-14T19:45:34Z","timestamp":1610653534000},"page":"2075-2084","source":"Crossref","is-referenced-by-count":30,"title":["Eculizumab discontinuation in atypical haemolytic uraemic syndrome: TMA recurrence risk and renal outcomes"],"prefix":"10.1093","volume":"14","author":[{"given":"Gema","family":"Ariceta","sequence":"first","affiliation":[{"name":"Department of Pediatric Nephrology, Vall d'Hebron Hospital, and the Autonomous University of Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fadi","family":"Fakhouri","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Immunology, CHU de Nantes, Nantes, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Sartz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Skane University Hospital, Lund University, Lund, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin","family":"Miller","sequence":"additional","affiliation":[{"name":"Alexion Pharmaceuticals, Inc., Boston, MA, USA*"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vasilis","family":"Nikolaou","sequence":"additional","affiliation":[{"name":"Parexel International Ltd, Uxbridge, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Cohen","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Nephrology, Columbia University Medical Center, New York, NY, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew M","family":"Siedlecki","sequence":"additional","affiliation":[{"name":"Department of 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