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Role of Autoimmunity and Autoinflammation in the Pathogenesis of Idiopathic Recurrent Pericarditis

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Abstract

Idiopathic recurrent pericarditis is the most common and troublesome complication of acute pericarditis affecting about one third of such patients. The pericardium may be involved in different systemic autoimmune diseases (i.e., systemic lupus erythematosus, rheumatoid arthritis, progressive systemic sclerosis, mixed connective tissue disease, Sjogren's Syndrome, polyarteritis, giant cell arteritis, other systemic vasculitides) either in a symptomatic form (usually during the active phase of the disease) or as asymptomatic pericardial effusion. Moreover, idiopathic recurrent pericarditis mimicks hereditary periodic fever syndromes (HPFSs). HPFSs are a group of disorders characterized by primary dysfunction of the innate immune system mostly caused by mutations of genes involved in the regulation or activation of the inflammatory response, without any apparent involvement of antigen-specific T cells or significant production of autoantibodies. These disorders usually manifest in the pediatric population, with onset ranging from the first hours to the first decade of life, however a limited number of patients experience disease onset during adulthood.

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Authors and Affiliations

  1. Interdepartmental Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Unit of Rheumatology, Policlinico Le Scotte, University of Siena, Siena, Italy

    Luca Cantarini, Maria Giuseppina Brizi & Mauro Galeazzi

  2. Cardiology Department, Ospedale Maria Vittoria, Turin, Italy

    Massimo Imazio

  3. Department of Evolutionary Biology, University of Siena, Siena, Italy

    Orso Maria Lucherini

  4. Internal Medicine, Ospedali Riuniti di Bergamo, Bergamo, Italy

    Antonio Brucato

  5. Department of Paediatrics, Rheumatology Unit, Anna Meyer Children’s Hospital and University of Florence, Florence, Italy

    Rolando Cimaz

  6. Rheumatology Unit, Policlinico Le Scotte, University of Siena, Viale Bracci 1, 53100, Siena, Italy

    Luca Cantarini

Authors
  1. Luca Cantarini
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  2. Massimo Imazio
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  3. Maria Giuseppina Brizi
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  4. Orso Maria Lucherini
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  5. Antonio Brucato
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  6. Rolando Cimaz
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  7. Mauro Galeazzi
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Correspondence to Luca Cantarini.

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Cantarini, L., Imazio, M., Brizi, M.G. et al. Role of Autoimmunity and Autoinflammation in the Pathogenesis of Idiopathic Recurrent Pericarditis. Clinic Rev Allerg Immunol 44, 6–13 (2013). https://doi.org/10.1007/s12016-010-8219-x

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  • DOI: https://doi.org/10.1007/s12016-010-8219-x

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