Síndrome Poliglandular Autoinmune: Revisión de una condición clínica subestimada

POLYGLANDULAR AUTOIMMUNE SYNDROME EPIDEMIOLOGICAL REVIEW OF AN UNDERESTIMATED CLINICAL CONDITION

Objetivo: Revisión al respecto del síndrome poli glandular autoinmune desde el punto de vista epidemiológico y clínico terapeutico. Materiales y Métodos: Se realizó una búsqueda detallada en la literatura científica durante los últimos 30 años sobre los términos Enfermedades Autoinmunes, Endocrinología, Genética, Asesoramiento Genético, síndrome poliglandular autoinmune a través de los motores de pubmed. Se seleccionaron las 50 referencias con mayor impacto en idioma ingles Resultados: Los síndromes autoinmunes poli glandulares son enfermedades multifactoriales con al menos dos endocrinopatías mediadas por autoinmunidad coexistentes. En la mayoría de las endocrinopatías el proceso autoinmune provoca una pérdida irreversible de la función glandular. El juvenil o tipo I se hereda de manera monogenética, mientras que ocurren varios polimorfismos de genes de susceptibilidad para los pacientes adultos. Ello es relevante para un diagnóstico oportuno en una etapa temprana es la detección de autoinmunidad poli glandular en pacientes con enfermedad autoinmune monoglandular y/o familiares de primer grado de pacientes con síndrome poli glandular autoinmune. En adultos la forma más prevalente es la combinación de diabetes tipo 1 con enfermedad tiroidea autoinmune. Conclusiones: Se recomienda la detección temprana de auto anticuerpos específicos y disfunción latente específica de órganos para alertar a quienes manejan este tipo de pacientes así como el asesoramiento genético oportuno.

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