Azizi G, Ziaee V, Tavakol M, Alinia T, Yazdai R, Mohammadi H, Abolhassani H, Aghamohammadi A
Department of Laboratory Medicine, Imam Hassan Mojtaba Hospital, Alborz University of Medical Sciences, Karaj, Iran.
Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
Scand J Immunol. 2017 Jan;85(1):13-29. doi: 10.1111/sji.12506.
Primary immunodeficiency diseases (PIDs) consist of a genetically heterogeneous group of immune disorders that affect distinct elements of the immune system. PID patients are more prone to infections and non-infectious complications, particularly autoimmunity. The concomitance of immunodeficiency and autoimmunity appears to be paradoxical and leads to difficulty in the management of autoimmune complications in PID patients. Therefore, management of autoimmunity in patients with PID requires special considerations because dysregulations and dysfunctions of the immune system along with persistent inflammation impair the process of diagnosis and treatment.
原发性免疫缺陷病（PID）是一组由基因异质性导致的免疫紊乱疾病，会影响免疫系统的不同组成部分。PID患者更容易发生感染和非感染性并发症，尤其是自身免疫性疾病。免疫缺陷与自身免疫同时存在似乎自相矛盾，导致PID患者自身免疫性并发症的管理存在困难。因此，PID患者自身免疫性疾病的管理需要特殊考虑，因为免疫系统的失调和功能障碍以及持续炎症会影响诊断和治疗过程。
