Document Detail

Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies.
MedLine Citation:
PMID:  23026949     Owner:  NLM     Status:  MEDLINE    
In the past decade, a large body of evidence has accumulated in support of the critical role of dysregulation of the alternative complement pathway in atypical haemolytic uraemic syndrome (aHUS) and C3 glomerulopathies. These findings have paved the way for innovative therapeutic strategies based on complement blockade, and eculizumab, a monoclonal antibody targeting the human complement component 5, is now widely used to treat aHUS. In this article, we review 28 case reports and preliminary data from 37 patients enrolled in prospective trials of eculizumab treatment for episodes of aHUS involving either native or transplanted kidneys. Eculizumab may be considered as an optimal first-line therapy when the diagnosis of aHUS is unequivocal and this treatment has the potential to rescue renal function when administered early after onset of the disease. However, a number of important issues require further study, including the appropriate duration of treatment according to an individual's genetic background and medical history, the optimal strategy to prevent post-transplantation recurrence of aHUS and a cost-efficacy analysis. Data regarding the efficacy of eculizumab in the control of C3 glomerulopathies are more limited and less clear, but several observations suggest that eculizumab may act on the most inflammatory forms of this disorder.
Julien Zuber; Fadi Fakhouri; Lubka T Roumenina; Chantal Loirat; Véronique Frémeaux-Bacchi;
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Publication Detail:
Type:  Journal Article; Review     Date:  2012-10-02
Journal Detail:
Title:  Nature reviews. Nephrology     Volume:  8     ISSN:  1759-507X     ISO Abbreviation:  Nat Rev Nephrol     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-10-24     Completed Date:  2013-04-01     Revised Date:  2013-11-25    
Medline Journal Info:
Nlm Unique ID:  101500081     Medline TA:  Nat Rev Nephrol     Country:  England    
Other Details:
Languages:  eng     Pagination:  643-57     Citation Subset:  IM    
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MeSH Terms
Antibodies, Monoclonal, Humanized / administration & dosage,  therapeutic use*
Complement Activation / drug effects
Hemolytic-Uremic Syndrome / drug therapy*,  immunology,  physiopathology,  surgery
Kidney / drug effects,  physiopathology
Kidney Transplantation
Meningitis, Meningococcal / complications,  prevention & control
Recurrence / prevention & control
Reg. No./Substance:
0/Antibodies, Monoclonal, Humanized; A3ULP0F556/eculizumab
M Buchler / ; S Burtey / ; D Chauveau / ; Y Delmas / ; G Deschênes / ; A Garnier / ; M Y Hourmant / ; A Karras / ; B Knebelmann / ; Y Lebranchu / ; B Legallicier / ; C Legendre / ; M Le Quintrec / ; B Moulin / ; P Niaudet / ; C Pouteil-Noble / ; F Provost / ; B Ranchin / ; E Rondeau / ; S Al-Akash / ; G Ardissimo / ; G Ariceta / ; J M Campistol / ; S Cataland / ; J C Davin / ; E M Dorresteijn / ; R Gruppo / ; K Halka / ; N Heyne / ; A L Lapeyrague / ; M Lozano / ; C Nester / ; J Nürnberger / ; R Ramos / ; C Reid / ; J J Kim / ; M Riedl / ; F Schaefer / ; G D Simonetti / ; M Weitz /

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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