Autoinflammatory syndromes and infections : pathogenetic and clinical implications

• Published in: Clinical and experimental rheumatology 2008, Vol.26 (1), p.S53-S61
• Main Authors: EFTHIMIOU, P, FLAVELL, R. A, DORIA, A, FURLAN, A, GASBARRINI, G, GAVA, A, KONE-PAUT, I, MANNA, R, PUNZI, L, SUTTERWALA, F. S, TOUITOU, I
• Format: Article
• Language: English
• Subjects: Autoimmune Diseases - immunology; Autoimmune Diseases - microbiology; Autoimmune Diseases - virology; Biological and medical sciences; Diseases of the osteoarticular system; Errors of metabolism; Familial Mediterranean Fever - immunology; Familial Mediterranean Fever - microbiology; Familial Mediterranean Fever - virology; Genetics; Human genetics; Humans; Infection - complications; Infection - immunology; Inflammation - immunology; Inflammation - microbiology; Inflammation - virology; Inflammatory joint diseases; Life Sciences; Malformations and congenital and or hereditary diseases involving bones. Joint deformations; Medical sciences; Metabolic diseases; Miscellaneous hereditary metabolic disorders; Nod Signaling Adaptor Proteins - immunology; Still's Disease, Adult-Onset - immunology; Still's Disease, Adult-Onset - microbiology; Still's Disease, Adult-Onset - virology
• ISSN: 0392-856X; 1593-098X

The autoinflammatory syndromes are a group of disorders characterized by recurrent episodes of seemingly unprovoked inflammation without significant levels of autoantobodies and antigen specific T cells. Although a direct association between defective innate immune responses to bacterial components and these diseases has not been formally established, much ongoing research is aimed towards confirmation of that hypothesis. This article will review recent advances in the study of a subset of NOD-like receptors (NLRs), which control the activation of caspase-1 through the assembly of a large protein complex called inflammasome. Moreover, we will review recent progresses in understanding of a range of autoinflammatory conditions in humans.