Abstract
Inherited autoinflammatory diseases are secondary to mutations of proteins playing a pivotal role in the regulation of the innate immunity leading to seemingly unprovoked episodes of inflammation. The understanding of the molecular pathways involved in these disorders has shed new lights on the pattern of activation and maintenance of the inflammatory response and disclosed new molecular therapeutic targets. Cryopyrin-associated periodic syndrome (CAPS) represents the prototype of an autoinflammatory disease. The study of the pathophysiological consequence of mutations in the cryopyrin gene (NLRP3) allowed the identification of intracellular pathways responsible for the activation and secretion of the potent inflammatory cytokine interleukin-1β (IL-1β). It became clear that several multi-factorial inflammatory conditions display a number of pathogenic and clinical similarities with inherited autoinflammatory diseases. The dramatic effect of interleukin-1 (IL-1) blockade in CAPS opened new perspectives for the treatment of other inherited and multi-factorial autoinflammatory disorders. Several IL-1 blockers are now available on the market. In this review we outline the more recent novelties in the treatment with different IL-1 blockers in inherited and multi-factorial autoinflammatory diseases.
Highlights
The Autoinflammatory Syndromes are a number of different conditions characterized by episodes of inflammation secondary to an activation of the innate arm of the immune response, in the absence of high-titer auto-antibodies or antigen-specific T cells [1]
Inherited autoinflammatory diseases are secondary to mutations of proteins playing a pivotal role in the regulation of the innate immunity leading to seemingly unprovoked episodes of inflammation
The term “Autoinflammatory diseases” was originally referred to a limited number of rare inherited diseases identified as periodic fevers
Summary
The Autoinflammatory Syndromes are a number of different conditions characterized by episodes of inflammation secondary to an activation of the innate arm of the immune response, in the absence of high-titer auto-antibodies or antigen-specific T cells [1]. The term “Autoinflammatory diseases” was originally referred to a limited number of rare inherited diseases identified as periodic fevers Under this term were gathered some monogenic diseases featured by periodic or recurrent episodes of systemic inflammation causing fever often associated with rash, serositis, lymphadenopathy, arthritis, and other clinical manifestations. These disorders were secondary to mutations of genes coding for proteins that play a pivotal role in the regulation of the inflammatory response. In physiological conditions IL-1β is in an inactive form and requires a series of intracellular events to be activated In normal conditions both IL-1α and IL-1β bind to type 1 IL-1 receptor (IL-1R1) and to the adaptor protein IL-1RAcP in order to trigger signal transduction (Figure 1). Other IL-1 blockers have been successively www.frontiersin.org
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