Monogenic polyautoimmunity in primary immunodeficiency diseases

Gholamreza Azizi, Reza Yazdani, Wiliam Rae, Hassan Abolhassani, Manuel Rojas, Asghar Aghamohammadi, Juan Manuel Anaya

Resultado de la investigación: Contribución a una revistaArtículo de revisiónrevisión exhaustiva

17 Citas (Scopus)

Resumen

Primary immunodeficiency diseases (PIDs) consist of a large group of genetic disorders that affect distinct components of the immune system. PID patients are susceptible to infection and non-infectious complications, particularly autoimmunity. A specific group of monogenic PIDs are due to mutations in genes that are critical for the regulation of immunological tolerance and immune responses. This group of monogenic PIDs is at high risk of developing polyautoimmunity (i.e., the presence of more than one autoimmune disease in a single patient) because of their impaired immunity. In this review, we discuss the mechanisms of autoimmunity in PIDs and the characteristics of polyautoimmunity in the following PIDs: IPEX; monogenic IPEX-like syndrome; LRBA deficiency; CTLA4 deficiency; APECED; ALPS; and PKCδ deficiency.

Idioma originalInglés estadounidense
Páginas (desde-hasta)1028-1039
Número de páginas12
PublicaciónAutoimmunity Reviews
Volumen17
N.º10
DOI
EstadoPublicada - oct 2018

All Science Journal Classification (ASJC) codes

  • Inmulogía y alergología
  • Inmunología

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