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. Author manuscript; available in PMC: 2015 Sep 1.
Published in final edited form as: Curr Opin Rheumatol. 2014 Sep;26(5):562–569. doi: 10.1097/BOR.0000000000000093

Figure 1. Self-perpetuating inflammatory cycles sustain enhanced systemic inflammation in hyperinflammatory syndromes.

Figure 1

The working model of pathogenesis for hyperinflammatory diseases occurs from amplifying feed-forward inflammatory cycles leading to downstream immunopathology. In MAS, an autoinflammatory trigger likely mediated through pattern recognition receptor activation of innate immune cells leads to the release of proinflammatory mediators. Effector cells amplify this proinflammatory cytokine cascade leading to hypercytokinemia. The combination of proinflammatory cytokines and continued activation of pattern recognition receptors (PRRs) leads to synergistic activation of innate immune cells driving an enhanced immunopathologic cycle (29). In FHL, viral infections stimulate antigen-presenting cells to activate cytotoxic T cells to release tissue-damaging effector molecules. Due to defective cytotoxicity, uncontrolled viral replication propagates robust innate immune cell antigen presentation perpetuating a vicious cycle of self-sustaining and self-enhancing systemic inflammation (30).