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Anti-Ro/SSA and the development of hematological malignancy in systemic autoimmune diseases
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Resumo(s)
Os auto-anticorpos estão associados a um número considerável doenças autoimunes. O anticorpo anti-Ro/SSA faz parte deste grupo vasto de anticorpos, podendo encontrar-se em doentes com Síndrome de Sjögren (SS) e Lupus Eritematoso Sistémico (LES), entre outros. O anticorpo anti-Ro tem como alvo o antigénio Ro que se compõe de duas proteínas diferentes, Ro60 e Ro52. Tem sido amplamente reconhecido na literatura que doentes com patologia autoimune como LES e SS têm um risco acrescido de desenvolver doenças hemato-oncológicas. Coincidentemente, tem sido descrita uma elevada incidência de anticorpos anti-Ro em doentes com patologia do foro hemato-oncológico, com diagnóstico autoimune prévio ou sem doença autoimune conhecida. Os potenciais mecanismos interligando a actividade dos anticorpos anti-Ro, o antigénio Ro e o desenvolvimento de doença hemato-oncológica permanecem ainda sob esclarecimento. O autor apresenta uma pequena série de doentes com patologia autoimune, positividade para anticorpos anti-Ro e desenvolvimento de doença hemato-oncológica, revendo a literatura mais recente e enfatizando o potencial papel da ativação da via de sinalização NF-kB como elo fisiopatológico entre doença autoimune, anti-Ro e malignidade hematológica.
Autoantibodies are associated with a considerable number of autoimmune diseases. Anti-Ro/SSA antibody is one of this vast group of antibodies and can be found in patients with Sjögren's Syndrome (SS) and Systemic Lupus Erythematosus (SLE), among other pathologies. The antibody anti-Ro targets the Ro antigen, composed of two different proteins, Ro52 and Ro60. It has been widely recognized in the literature that patients with autoimmune pathology such as SLE and SS have an increased risk of developing hemato-oncological diseases. Coincidentally, a high incidence of anti-Ro antibodies has been described in patients with hemato-oncological pathology, with a previous autoimmune diagnosis or without known autoimmune disease. The potential mechanisms linking the activity of anti-Ro antibodies, the Ro antigen and the development of hemato-oncological disease remain under elucidation. The author presents a small series of patients with autoimmune pathology, positivity for anti-Ro antibodies and development of hemato-oncological disease, reviewing the most recent literature and emphasizing the potential role of activation of the NF-kB signaling pathway as a pathophysiological link between disease autoimmune, anti-Ro and hematologic malignancy.
Autoantibodies are associated with a considerable number of autoimmune diseases. Anti-Ro/SSA antibody is one of this vast group of antibodies and can be found in patients with Sjögren's Syndrome (SS) and Systemic Lupus Erythematosus (SLE), among other pathologies. The antibody anti-Ro targets the Ro antigen, composed of two different proteins, Ro52 and Ro60. It has been widely recognized in the literature that patients with autoimmune pathology such as SLE and SS have an increased risk of developing hemato-oncological diseases. Coincidentally, a high incidence of anti-Ro antibodies has been described in patients with hemato-oncological pathology, with a previous autoimmune diagnosis or without known autoimmune disease. The potential mechanisms linking the activity of anti-Ro antibodies, the Ro antigen and the development of hemato-oncological disease remain under elucidation. The author presents a small series of patients with autoimmune pathology, positivity for anti-Ro antibodies and development of hemato-oncological disease, reviewing the most recent literature and emphasizing the potential role of activation of the NF-kB signaling pathway as a pathophysiological link between disease autoimmune, anti-Ro and hematologic malignancy.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2021
Palavras-chave
Lupus eritematoso sistémico Síndrome de Sjögren Doença hematológica maligna Anti-Ro/SSA NF-kB