Case report: Short-term eculizumab use in atypical HUS associated with Lemierre's syndrome and post-infectious glomerulonephritis

Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by genetic abnormalities, infections, autoimmune diseases, drugs, and malignancies. Anti-C5 monoclonal antibody eculizumab is the mainstay of treatment of aHUS caused by the genetic defects of the alternative complement pathway. Howe...

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Bibliographic Details
Published inFrontiers in medicine Vol. 10; p. 1167806
Main Authors Sadiq, Sanober, Urisman, Anatoly, Cil, Onur
Format Journal Article
LanguageEnglish
Published Switzerland Frontiers Media S.A 03.05.2023
Subjects
atypical HUS
eculizumab discontinuation
Eikenella
Fusobacterium
Medicine
prognosis
eculizumab discontinuation
Eikenella
Fusobacterium
prognosis
atypical HUS
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Summary:Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by genetic abnormalities, infections, autoimmune diseases, drugs, and malignancies. Anti-C5 monoclonal antibody eculizumab is the mainstay of treatment of aHUS caused by the genetic defects of the alternative complement pathway. However, the utility of eculizumab in non-genetic forms of aHUS and the timing of treatment discontinuation remain controversial. Here, we report successful short-term eculizumab use in two young adult patients with aHUS due to rare infectious and autoimmune etiologies: Lemierre's syndrome and post-infectious glomerulonephritis, respectively. Eculizumab was rapidly discontinued in both patients with no aHUS recurrence during long-term follow-up. Considering its favorable safety profile with appropriate meningococcal prophylaxis, eculizumab can be considered as a treatment option for non-genetic aHUS.
Bibliography:Edited by: Gian Marco Ghiggeri, Giannina Gaslini Institute (IRCCS), Italy
Reviewed by: Erica Daina, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, Italy; Eleni Gavriilaki, G. Papanikolaou General Hospital, Greece
ISSN:2296-858X
2296-858X
DOI:10.3389/fmed.2023.1167806