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Resumen de Terapias innovadoras para el manejo de la enfermedad de Behçet refractaria

Belén Atienza Mateo

  • español

    La enfermedad de Behçet (EB) es una enfermedad autoinmune, inflamatoria crónica, englobada dentro del grupo de las vasculitis sistémicas de vaso de tamaño variable, caracterizada por la presencia de úlceras orales junto con manifestaciones sistémicas, incluyendo afectación ocular, cutánea, gastrointestinal, neurológica, vascular y/o articular. Su prevalencia es mayor en los países mediterráneos que conformaban antiguamente la “Ruta de la seda”. La naturaleza remitente-recurrente de la EB y las diferencias en la afectación de los distintos órganos y sistemas, así como en el curso de la enfermedad según sexo, edad y raza, hacen que el tratamiento deba ser individualizado. Desafortunadamente, existen pacientes que no responden o no toleran las terapias inmunosupresoras convencionales. Los trabajos realizados en esta Tesis concluyen que el empleo de agentes biológicos anti-TNF, tanto infliximab como adalimumab, es efectivo en pacientes con EB y uveítis grave/ refractaria. Por otro lado, apremilast podría ser beneficioso para tratar las úlceras orogenitales que no respondan al tratamiento convencional, incluso en combinación con otros inmunosupresores. Finalmente, tocilizumab constituye una alternativa eficaz y segura en pacientes con fenotipo clínico ocular y neurológico, especialmente en los casos más complicados.

    Behçet's disease (BD) is a chronic inflammatory autoimmune disease, within the group of variable-vessel vasculitis, characterized by the presence of recurrent oral ulcers with systemic manifestations, including ocular, cutaneous, gastrointestinal, neurological, vascular and/or joint involvement. Its prevalence is higher in the Mediterranean countries along the ancient “Silk Road”. The relapsing-remitting nature of BD and the different organ involvement, as well as the differences in the course of the disease by sex, age and race, require individualized treatments. Unfortunately, there are patients who do not respond to or do not tolerate conventional immunosuppressive therapies. The studies carried out in this Thesis conclude that the use of biological anti-TNF agents, both infliximab and adalimumab, is effective in patients with BD and severe/ refractory uveitis. On the other hand, apremilast could be beneficial to treat orogenital ulcers that do not respond to conventional treatment, even in combination with other immunosuppressants. Finally, tocilizumab is an effective and safe alternative in patients with ocular and neurological phenotypes, especially in the most complicated cases.

  • English

    Behçet's disease (BD) is a chronic inflammatory autoimmune disease, within the group of variable-vessel vasculitis, characterized by the presence of recurrent oral ulcers with systemic manifestations, including ocular, cutaneous, gastrointestinal, neurological, vascular and/or joint involvement. Its prevalence is higher in the Mediterranean countries along the ancient “Silk Road”. The relapsing-remitting nature of BD and the different organ involvement, as well as the differences in the course of the disease by sex, age and race, require individualized treatments. Unfortunately, there are patients who do not respond to or do not tolerate conventional immunosuppressive therapies. The studies carried out in this Thesis conclude that the use of biological anti-TNF agents, both infliximab and adalimumab, is effective in patients with BD and severe/ refractory uveitis. On the other hand, apremilast could be beneficial to treat orogenital ulcers that do not respond to conventional treatment, even in combination with other immunosuppressants. Finally, tocilizumab is an effective and safe alternative in patients with ocular and neurological phenotypes, especially in the most complicated cases.


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