Immunopathological Role Of Neutrophil Extracellular Traps (Nets) In Rare Autoimmune And Autoinflammatory Diseases: A Scoping Review

Abstract

Neutrophil extracellular traps (NETs) are an important of the innate immune system that functions to protect the body from pathogens. However, when their formation and clearance processes are disrupted, NETs can contribute to the development of various autoimmune diseases, including rare conditions. Therefore, this study aims to describe the immunopathological role of NETs in rare autoimmune and autoinflammatory diseases through a scoping review approach. A literature search was conducted in the Scopus, PubMed, and Web of Science databases following the PRISMA-ScR guidelines. From a total of 525 identified articles, 10 studies met the inclusion criteria after the selection process and were analyzed further. The results show that the role of NETs can be classified into three main mechanisms. First, enhanced NET formation (enhanced NETosis), triggers the release of autoantigens and the formation of immune complexes. Second, impaired clearance, leads to the accumulation of NETs and prolongs the inflammatory process. Third, the interaction of NETs with other immune system components, including the complement system, which further amplifying the inflammatory response. These three mechanisms are interconnected and form a continuous inflammatory cycle, contributing to tissue damage in rare autoimmune diseases. These findings indicate that NETs represent a potential target for the development of more specific therapies in the future.