Consenso latinoamericano de síndrome hemolítico urémico atípico
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Fecha
2025
Autores
Nieto-Ríos, John F.
Bello Márquez, Diana Carolina
MORALES-BUENROSTRO, LUIS
Musalem, Pilar
Vaisbich, Maria Helena
Fortunato, Rita M.
Gómez-Navarro, Benjamín
Jiménez-Acosta, Darío
Maldonado , Rafael Alberto
Trimarchi, Hernán
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Sociedad Latinoamericana de Nefrología e Hipertensión
Resumen
El síndrome hemolítico urémico atípico primario es una microangiopatía trombótica relacionada con una alteración en la
regulación de la vía alterna del complemento, caracterizada por daño endotelial difuso, hemólisis microangiopática y compromiso
orgánico multisistémico, ocasionando una alta morbimortalidad. Su diagnóstico diferencial incluye la púrpura trombocitopénica
trombótica, el síndrome hemolítico urémico relacionado con la toxina Shiga y las microangiopatías trombóticas
secundarias. En su evaluación son fundamentales la medición de la actividad de la enzima ADAMTS-13, la determinación
de la toxina Shiga en materia fecal o con pruebas moleculares que identifiquen las bacterias productoras de esta toxina, el
estudio de enfermedades sistémicas que causan microangiopatías trombóticas y la realización de pruebas genéticas de la
regulación de la vía alterna del complemento. El tratamiento de elección es la inhibición de la fracción C5 del complemento
con fármacos como el eculizumab y el ravulizumab. El pronóstico depende de un diagnóstico temprano y de un tratamiento
oportuno. En Latinoamérica, este síndrome ha sido escasamente estudiado y hay pocos consensos que unifiquen
los criterios de evaluación y manejo.
Primary atypical hemolytic uremic syndrome is a thrombotic microangiopathy associated with altered regulation of the alternate complement pathway. It is characterized by diffuse endothelial damage, microangiopathic hemolysis, and multisystem organ involvement, causing high morbidity and mortality. Its differential diagnosis includes thrombotic thrombocytopenic purpura, Shiga toxin-associated hemolytic uremic syndrome, and secondary thrombotic microangiopathies. Its evaluation includes measuring ADAMTS-13 enzyme activity, determining Shiga toxin in fecal matter, or using molecular tests to identify the bacteria that produce this toxin. Its evaluation also includes the study of systemic diseases that cause thrombotic microangiopathies and performing genetic testing for regulation of the alternate complement pathway. The treatment of choice is inhibition of the C5 fraction of complement with drugs such as eculizumab and ravulizumab. The prognosis depends on early diagnosis and timely treatment. In Latin America, this syndrome has been poorly studied, and there is little consensus that unifies evaluation and management criteria.
Primary atypical hemolytic uremic syndrome is a thrombotic microangiopathy associated with altered regulation of the alternate complement pathway. It is characterized by diffuse endothelial damage, microangiopathic hemolysis, and multisystem organ involvement, causing high morbidity and mortality. Its differential diagnosis includes thrombotic thrombocytopenic purpura, Shiga toxin-associated hemolytic uremic syndrome, and secondary thrombotic microangiopathies. Its evaluation includes measuring ADAMTS-13 enzyme activity, determining Shiga toxin in fecal matter, or using molecular tests to identify the bacteria that produce this toxin. Its evaluation also includes the study of systemic diseases that cause thrombotic microangiopathies and performing genetic testing for regulation of the alternate complement pathway. The treatment of choice is inhibition of the C5 fraction of complement with drugs such as eculizumab and ravulizumab. The prognosis depends on early diagnosis and timely treatment. In Latin America, this syndrome has been poorly studied, and there is little consensus that unifies evaluation and management criteria.
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Palabras clave
Microangiopatías trombóticas, Síndrome hemolítico urémico atípico, Intercambio plasmático, Eculizumab, Ravulizumab
