Novel insights into the role of inflammasomes in autoimmune and metabolic rheumatic diseases
Inflammasomes are large intracellular complexes that induce inflammation in response to exogenous and endogenous damage signals. They regulate production and release of the proinflammatory cytokines IL-1β and IL-18, playing a defensive role against infections. Inflammasomes have also been involved in the pathogenesis of a wide range of autoinflammatory conditions that are caused by dysregulation of the IL-1 pathway, such as cryopyrinopathies and hereditary periodic fever syndromes. On top of that, research in recent years suggests that defects in inflammasome regulation and signaling associate with a number of autoimmune diseases such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis and others. In this review, we describe the inflammasome and mechanisms that trigger it, provide a brief review of autoinflammatory disorders and discuss the current understanding and emerging data from experimental and clinical studies for the role of the innate immune system and inflammasomes in the biology and pathogenesis of systemic autoimmune diseases.
KeywordsInflammasomes Systemic rheumatic disease Systemic sclerosis Systemic lupus erythematosous Rheumatoid arthritis Gout
The study received no funding.
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Conflict of interest
The authors declare no conflict of interest.
This article does not contain any studies with human participants or animals performed by any of the authors.
- 12.Lucherini OM, Rigante D, Sota J (2018) Updated overview of molecular pathways involved in the most common monogenic autoinflammatory diseases. Clin Exp Rheumatol Suppl 110:3–9Google Scholar
- 32.Reddy S, Jia S, Geoffrey R et al (2009) An autoinflammatory disease due to homozygous deletion of the IL1RN locus. N. Engl. J Med 360:2438–2444Google Scholar
- 40.Younis AA (2017) Crowned dens syndrome as a cause of acute neck pain: a case report and review of the literature. Mediterr J Rheumatol 28:53–57Google Scholar
- 49.Bhattacharyya S, Varga J (2015) Emerging roles of innate immune signaling and toll-like receptors in fibrosis and systemic sclerosis. Curr Rheumtol Rep 17:474Google Scholar
- 71.Mathews RJ, Robinson JI, Battellino M et al (2014) Evidence of NLRP3-inflammasome activation in rheumatoid arthritis (RA); genetic variants within the NLRP3-inflammasome complex in relation to susceptibility to RA and response to anti-TNF treatment. Ann Rheum Dis 73:1202–1210CrossRefPubMedGoogle Scholar