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Focus 2-2 (FS 2-2) : Auto-inflammation vs autoimmunity
최지호 ( Jee Ho Choi )
UCI I410-ECN-0102-2015-500-002021461
This article is 4 pages or less.

Autoimmune disease is defined as disease in which the pathology is caused by adaptive immune reponses to self/auto antigen. Autoimmunity is the failure of an organism in recognizing its own constituent parts as self, which allows an immune response against its own cells and tissues. Any disease that results from such an aberrant immune response is termed an autoimmune disease. Autoinflammatory disease is defined as disease due to unregulated inflammation in the absence of infection. Autoinflammatory syndrome has been attributed to the group of disorders characterized by unexplained recurrent attacks of inflammation without any evidence that this process is related to rejection of autoantigen. Autoimmunity and autoinflammation share some common characteristics: both lead to self-directed inflammation, but with different mechanisms. Although autoimmunity involves adaptive immune activation, autoinflammation involves innate immune activation. Autoinflammation is genetically related to perturbations of innate immune function, including proinflammatory cytokine signaling abnormalities, or bacterial sensing, or local tissue abnormalities. The clinical and pathophysiologic expressions of autoinflammation are determined by cells of the innate immune system, including neutrophils and macrophages or nonimmune cells. Autoinflammatory diseases (AIDs), concept recently introduced in the medical literature, are a category of illnesses characterized by seemingly unprovoked episodes of inflammation, without high-titer autoantibodies or antigen-specific T cells, in which, as in the autoimmune diseases, the capability of self-recognition is disturbed, initiating an attack on the body’s own tissues. The distorted and abnormal response generates a cellular network with the implicated chemokine chain reaction that, in turn, generates an inflammatory response. Inflammasomes are intracytosolic multiprotein innate immune complexes that recognize pathogen-associated molecular patterns. Recognition of pathogen-associated molecular patterns is followed by production of the proinflammatory cytokines IL-1 and IL-18. Inflammasomes are molecular platforms activated upon cellular infection or stress that trigger the maturation of proinflammatory cytokines such as IL-1 to engage innate immune defenses. Inflammasome is a multiprotein oligomer consisting of caspase 1, PYCARD, NALP and sometimes caspase 5 (also known as caspase 11 or ICH-3). The inflammasome promotes the maturation of the inflammatory cytokines IL-1β and IL-18 and is responsible for activation of inflammatory processes, and has been shown to induce cell pyroptosis, a process of programmed cell death distinct from apoptosis. Masters et al. published an article in which 6 categories of AIDs were defined: 1. IL-1 activation disorders (inflammasomopathies), 2. NF-κB activation syndromes, 3. protein misfolding disorders, 4. complement regulatory diseases, 5. disturbances in cytokine signaling, 6. macrophage activation syndromes. AIDs are characterized by spontaneous activation of cells of innate immunity in the absence of ligands. Autoantibodies are usually not found. The neutrophils, widely considered an important component of autoinflammatory pathogenesis, have been found in early psoriatic lesions. Their function is modulated by T lymphocytes and by monocytes. As a common factor, neutrophil migration is observed in autoinflammatory cryopyrin-associated periodic syndrome (CAPS) as well as reported on common inflammatory keratoses like psoriasis. The molecular pathogenesis is associated in both conditions with an abnormal regulation of the innate immune response and Th17 cell differentiation via IL-1 signaling. Pustular psoriasis, a clinical variety of psoriasis that is now being considered as a separate entity and included in the latest classification of AIDs. GPP shows several features in common with autoinflammatory disorders, including an intermittent course of disease, fever, neutrophilia, and signs of systemic inflammation.

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