About this Research Topic
The disease is a multifactorial process with both T and B lymphocytes playing important roles at all stages, multiple T and B-cell abnormalities’ with atypical cytokine profiles, loss of central tolerance, as well as defects in antigen presenting cells have all been implicated. Among the candidate genes identified, the strongest association exists with complete deficiency of C1q and C4, 90% and 80%, respectively with weaker associations seen with deficiencies of C1r, C1s, and C2. Forming an interface between activated complement components and various cell phenotypes are complement regulators and receptors. Despite the fact that no other known genetic deficiency confers such a high penetrance of human systemic autoimmune disease as complete C1q and C4 deficiency, our knowledge of the role played by these proteins and their receptors in cellular homeostasis and in patients with SLE is incomplete, especially in regards to the effects of C4 isotypes and their various alleles. Complement fragments bound to each other and within an immune complex could simultaneously ligate more than one receptor thus altering that cell’s behavior or serve as a bridge to initiate crosstalk between cells.
Hence, this Research Topic will highlight the multiple links existing between the early components of complement and immune cell responses as they might relate to systemic autoimmune disease. Since the mechanism underlying the relationship between these complement deficiencies and SLE is still unclear we will also entertain novel hypothetical papers in this area.
Important Note: All contributions to this Research Topic must be within the scope of the section and journal to which they are submitted, as defined in their mission statements. Frontiers reserves the right to guide an out-of-scope manuscript to a more suitable section or journal at any stage of peer review.