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Current Immunology Reviews

Eiditor-in-Chief

ISSN (Print): 1573-3955
ISSN (Online): 1875-631X

Review Article

Clinical Approach to Systemic Autoinflammatory Disorders: Classification, Disease Phenotypes and Management

Author(s): Tiago Borges*

Volume 14 , Issue 2 , 2018

Page: [105 - 119] Pages: 15

DOI: 10.2174/1573395514666180621152753

Price: $58

Abstract

Autoinflammatory Disorders (AIDs) can be either systemic or organ-specific. Systemic AIDs are associated with a primary dysfunction of the innate immunity that results in clinical signs of inflammation and/or elevated serum levels of acute-phase reactants. AIDs often present with rash, serositis, arthritis and/or aphthosis. Hereditary periodic fever syndromes, such as Familial Mediterranean Fever (FMF) and Tumor Necrosis Factor Receptor-Associated Periodic Syndrome (TRAPS), constitute the prototype of systemic AIDs. However, these also include many other disorders that do not follow the typical relapse-remitting pattern of unprovoked recurrent febrile episodes with intercalated periods of general well-being that can also result in severe complications such as amyloidosis. In this review, previous classification systems of AIDs, either clinical or pathophysiological/functional-based, have been revised and systemic AIDs are individually described. Also, a simplified classification based on clinical phenotypes that differentiates systemic from organ-specific AIDs is discussed.

Keywords: autoinflammatory disorders, inflammasome, periodic fever syndromes, fever, inflammation, systemic.

Graphical Abstract

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