LAUSR

Interleukin-1 receptor-associated kinase 4 (IRAK4) is a serine/ threonine kinase involved in the signaling pathway stimulated by Toll-like receptors (TLRs) and Interleukin-1 (IL-1). Its function is crucial to the body’s innate immunity where its recruitment by adaptor proteins, such as MYD88, leads to the release of pro-inflammatory cytokines involved in the nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κβ) pathway. Appreciation for the role of IRAK4 in the body’s natural defense has grown over the years, and work has progressed to explore the kinase’s role in treating autoimmune diseases. In contrast, deficient IRAK4 activation can lead to serious systemic sequelae such as bacterial infections and bacteremia. Less is known about ocular manifestations in the setting of IRAK4 deficiency. While endogenous endophthalmitis has been described, non-infectious inflammation (uveitis) is rarely reported.