التفاصيل البيبلوغرافية
| العنوان: |
Microangiopatía trombótica secundaria y eculizumab: una opción terapéutica razonable. (Spanish) |
| العنوان البديل: |
Secondary thrombotic microangiopathy and eculizumab: A reasonable therapeutic option. (English) |
| المؤلفون: |
Román, Elena, Mendizábal, Santiago, Jarque, Isidro, De La Rubia, Javier, Sempere, Amparo, Morales, Enrique, Praga, Manuel, Ávila, Ana, Luis Górriz, José |
| المصدر: |
Nefrologia; 2017, Vol. 37 Issue 5, p478-491, 14p |
| الملخص (بالإنجليزية): |
Understanding the role of the complement system in the pathogenesis of atypical haemolytic uraemic syndrome and other thrombotic microangiopathies (TMA) has led to the use of anti-complement therapy with eculizumab in these diseases, in addition to its original use in patients with paroxysmal nocturnal haemoglobinuria andatypical haemolytic uraemic syndrome. Scientific evidence shows that both primary and secondary TMAs with underlying complement activation are closely related. For this reasons, control over the complement system is a therapeutic target. There are 2 scenarios in which eculizumab is used in patients with TMA: primary or secondary TMA that is difficult to differentiate (including incomplete clinical presentations) and complement-mediated damage in various processes in which eculizumab proves to be efficacious. This review summarises the evidence on the role of the complement activation in the pathophysiology of secondary TMAs and the efficacy of anticomplement therapy in TMAs secondary to pregnancy, drugs, transplant, humoral rejection, systemic diseases and glomerulonephritis. Although experience is scarce, a good response to eculizumab has been reported in patients with severe secondary TMAs refractory to conventional treatment. Thus, the role of the anti-complement therapy as a new treatment option in these patients should be investigated. [ABSTRACT FROM AUTHOR] |
| Abstract (Spanish): |
Elconocimiento del papel del complemento en la patogenia del síndrome hemolítico uré-mico atípico y otras microangiopatías trombóticas (MAT) ha fomentado el desarrollo de la terapia anticomplemento con eculizumab más allá de su indicación original en la hemo-globinuria paroxística nocturna y en el síndrome hemolítico urémico atípico. La evidencia científica demuestra un estrecho límite entre MAT primarias y secundarias con activación del complemento subyacente en ambas. Por ello, el control del complemento se convierte en una diana terapéutica. El uso de eculizumab en MAT secundarias contempla 2 escenarios: diagnóstico diferencial difícil entre MAT primaria y secundaria (incluidos cuadros clínicos incompletos) o daño por complemento en procesos distintos, donde se demuestra la eficacia del tratamiento. Esta revisión es una síntesis de la evidencia científica sobre el papel de la activación del complemento en la fisiopatología de las MAT secundarias y la eficacia de la terapia anticomplemento en MAT asociadas a embarazo, fármacos, trasplante, rechazo humoral, enfermedades sistémicas y glomerulonefritis. La experiencia es aún limitada, pero la respuesta a eculizumab en pacientes con MAT secundarias graves y refractarias al tratamiento convencional abre una puerta a la investigación de la terapia anticomplemento como nueva opción terapéutica. [ABSTRACT FROM AUTHOR] |
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