The plethora, clinical manifestations and treatment options of autoimmunity in patients with primary immunodeficiency.

Baris H. E. , Kiykim A., Nain E., ÖZEN A. O. , Karakoc-Aydiner E. , BARIŞ S.

Turk pediatri arsivi, vol.51, no.4, pp.186-192, 2016 (Journal Indexed in ESCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 51 Issue: 4
  • Publication Date: 2016
  • Doi Number: 10.5152/turkpediatriars.2016.3928
  • Title of Journal : Turk pediatri arsivi
  • Page Numbers: pp.186-192
  • Keywords: Autoimmunity, autoimmune hemolytic anemia, inflammatory bowel disease, primary immunodeficiency, PRIMARY IMMUNE-DEFICIENCY, X-LINKED SYNDROME, CUTANEOUS GRANULOMAS, T-CELLS, POLYENDOCRINOPATHY, DYSREGULATION, ENTEROPATHY, DISEASE, DISORDERS, MUTATIONS


Aim: Although the association between primary immunodeficiency and autoimmunity is already well-known, it has once again become a topic of debate with the discovery of newly-defined immunodeficiencies. Thus, investigation of the mechanisms of development of autoimmunity in primary immunodefficiency and new target-specific therapeutic options has come to the fore. In this study, we aimed to examine the clinical findings of autoimmunity, autoimmunity varieties, and treatment responses in patients who were genetically diagnosed as having primary immunodeficiency.