Complement inhibitor eculizumab in atypical hemolytic uremic syndrome.
Clin J Am Soc Nephrol
; 4(8): 1312-6, 2009 Aug.
Article
em En
| MEDLINE
| ID: mdl-19556379
ABSTRACT
BACKGROUND AND OBJECTIVES:
Atypical hemolytic uremic syndrome (aHUS) is associated with a congenital or acquired dysregulation of the complement alternative pathway that leads to continuous complement activation on host cells causing inflammation and damage. Eculizumab, a humanized mAb against complement protein C5, inhibits activation of the terminal complement pathway. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS We report an adolescent with relapsing unclassified aHUS. On admission, a high plasma creatinine level indicated a poor prognosis, and hemodialysis had to be started. Plasma exchanges were initially effective against the microangiopathic hemolytic activity and allowed a temporary improvement of renal function with termination of hemodialysis after 7 wk. Subsequently, plasma exchanges (three times per week) failed to prevent ongoing aHUS activity and progressive renal failure. After 12 wk, aHUS treatment was switched to eculizumab.RESULTS:
Eculizumab was effective in terminating the microangiopathic hemolytic process in two aHUS relapses; however, after normalization of complement activity, aHUS recurred and ultimately led to anuric end-stage renal failure.CONCLUSIONS:
In this patient, complement inhibition by eculizumab temporarily terminated the microangiopathic hemolytic activity. Nevertheless, renal damage as a result of preceding and subsequent aHUS activity resulted in end-stage renal failure; therefore, therapeutic success may depend on early administration of eculizumab. The optimal duration of treatment may be variable and remains to be determined.
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Ativação do Complemento
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Síndrome Hemolítico-Urêmica
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Fatores Imunológicos
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Falência Renal Crônica
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Anticorpos Monoclonais
Tipo de estudo:
Prognostic_studies
Limite:
Adolescent
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Humans
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Male
Idioma:
En
Revista:
Clin J Am Soc Nephrol
Assunto da revista:
NEFROLOGIA
Ano de publicação:
2009
Tipo de documento:
Article
País de afiliação:
Áustria