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Whereas in secondary antiphospholipid syndrome, e.g. to systemic lupus erythematosus, there is emerging evidence of a benefit from complement blockade with eculizumab, optimal treatment of primary antiphospholipid syndrome-associated thrombotic microangiopathy is currently unknown. We report the case of a 36-year-old male patient with primary antiphospholipid syndrome-associated thrombotic microangiopathy, presenting with a clinical picture of atypical hemolytic-uremic syndrome with frequent relapses, treated with eculizumab (four 900\u00a0mg weekly doses followed by 1200\u00a0mg fortnightly infusions) leading to resolution of hemolysis, long-term remission and partial kidney function recovery (peak serum creatinine 3.8\u00a0mg\/dL, decreased and stabilized around 2.5\u00a0mg\/dL) over a follow up period of over 2 years.<\/jats:p>","DOI":"10.1007\/s40620-023-01789-4","type":"journal-article","created":{"date-parts":[[2023,10,17]],"date-time":"2023-10-17T10:02:16Z","timestamp":1697536936000},"page":"1141-1145","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Thrombotic microangiopathy due to primary antiphospholipid syndrome: successful treatment with eculizumab"],"prefix":"10.1093","volume":"37","author":[{"given":"Chiara","family":"Pala","sequence":"first","affiliation":[{"name":"Nefrologia, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy 1"},{"name":"Dipartimento di Medicina e Chirurgia, Universit\u00e0 degli Studi di Parma, Parma, Italy 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabetta","family":"Parenti","sequence":"additional","affiliation":[{"name":"Nefrologia, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy 1"},{"name":"Dipartimento di Medicina e Chirurgia, Universit\u00e0 degli Studi di Parma, Parma, Italy 2"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Vizzini","sequence":"additional","affiliation":[{"name":"Dipartimento di Medicina e Chirurgia, Universit\u00e0 degli Studi di Parma, Parma, Italy 2"},{"name":"Laboratorio di Immunopatologia Renale \u201cLuigi Migone\u201d, Universit\u00e0 degli Studi di Parma, Parma, Italy 3"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Davide","family":"Gianfreda","sequence":"additional","affiliation":[{"name":"Nefrologia e Dialisi, Ospedale \u201cSanta Caterina Novella\u201d, Galatina, Lecce, Italy 4"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8886-4329","authenticated-orcid":false,"given":"Giovanni Maria","family":"Rossi","sequence":"additional","affiliation":[{"name":"Nefrologia, Azienda Ospedaliero-Universitaria di Parma, Parma, Italy 1"},{"name":"Dipartimento di Medicina e Chirurgia, Universit\u00e0 degli Studi di Parma, Parma, Italy 2"},{"name":"Laboratorio di Immunopatologia Renale \u201cLuigi Migone\u201d, Universit\u00e0 degli Studi di Parma, Parma, Italy 3"},{"name":"Nefrologia e Dialisi, Ospedale \u201cSanta Caterina Novella\u201d, Galatina, Lecce, Italy 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