title: The role of the inflammasome in patients with autoinflammatory diseases.
abstract: Autoinflammatory diseases are disorders of the innate immune system, characterized by systemic inflammation often driven by inflammasomes, and independent of infection and autoreactive antibodies or antigen-specific T cells. These diseases are increasingly recognized as disorders of immune dysregulation, presenting with a constellation of fevers, rashes, and mucosal symptoms in many cases, which suggests that the allergist/immunologist is the appropriate specialist for these patients. However, many practicing physicians are unaware of these disorders in their pediatric and adult patient populations, leading to substantial delays in diagnosis. Recognizing autoinflammatory disease symptom patterns, performing appropriate diagnostic tests, and instituting early effective therapy are essential to reduce morbidity and mortality in these patients. This review will focus on understanding the molecular basis of inflammasomes, recognizing the distinguishing features of the classic autoinflammatory disorders, and appreciating the treatment modalities available.
authors.affiliation: Division of Pediatric Allergy, Immunology, and Rheumatology, University of California at San Diego, and Rady Children's Hospital of San Diego, La Jolla, Calif. Electronic address: hahoffman@ucsd.edu.
authors.forename: Hal M
authors.initials: HM
authors.lastname: Hoffman
authors.affiliation: Division of Pediatric Allergy, Immunology, and Rheumatology, University of California at San Diego, and Rady Children's Hospital of San Diego, La Jolla, Calif.
authors.forename: Lori
authors.initials: L
authors.lastname: Broderick
mesh_terms: D000818:Animals; D000893:Anti-Inflammatory Agents / Q000494:pharmacology / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism* / Q000628:therapy; D015551:Autoimmunity; D055808:Drug Discovery; D020022:Genetic Predisposition to Disease; D006801:Humans; D007113:Immunity, Innate; D007155:Immunologic Factors / Q000494:pharmacology / Q000627:therapeutic use; D056747:Immunomodulation; D058847:Inflammasomes / Q000378:metabolism*; D007249:Inflammation / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism* / Q000628:therapy; D058990:Molecular Targeted Therapy; D010641:Phenotype; D015398:Signal Transduction
chemical_list: D000893:Anti-Inflammatory Agents; D007155:Immunologic Factors; D058847:Inflammasomes
keywords: Autoinflammation; inflammasome; innate immunity