Autoimmunity and Inborn Errors of Immunity: Two Faces of the Same Coin!

Abstract

Autoimmune disorders are a heterogeneous group of disorders characterized by immune dysregulation and are associated with a loss of tolerance to self-antigens. Autoimmunity in patients with inborn errors of immunity (IEI) has always been a puzzling phenomenon! Autoimmunity may be the first clinical presentation or sequel in patients with IEI. In this paper, we discuss the mechanisms of autoimmunity in IEI and present a few clinical cases highlighting the need to consider an IEI in patients presenting with autoimmunity and infections, polyautoimmunity, and polyendocrinopathy.

Keywords

• autoimmunity
• immune deficiency
• immune dysregulation