Síndrome Poliglandular Autoinmune: Revisión de una condición clínica subestimada

Abstract

Objective: Literature review about the autoimmune polyglandular syndrome from the epidemiological and clinical-therapeutic point of view. Materials and Methods: A detailed scientific literature searching was done from the last 30 years including the terms Autoimmune Diseases, Endocrinology, Genetics, Genetic Counseling, autoimmune polyglandular syndrome through the Pubmed database. 50 references in English of the greatest impact were selected. Results: Polyglandular autoimmune syndromes are multifactorial diseases with at least two coexisting autoimmune-mediated endocrinopathies. In most endocrinopathies, the autoimmune process causes an irreversible loss of glandular function. Juvenile or type I is monogenetically inherited, while in adult patients several susceptibility polymorphism genes occur. This is relevant for a timely diagnosis at early stages in the detection of polyglandular autoimmunity in patients with monogranular autoimmune disease and/or patients with polyglandular autoimmune syndrome with first-degree relatives. In adults, the most prevalent form is the combination of type 1 diabetes with autoimmune thyroid disease. Conclusions Early detection of specific autoantibodies and specific latent risk organ dysfunction is recommended to alert those who manage this type of patients, as well as an appropriate genetic counseling.