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Diagnosis and treatment of autoinflammatory diseases in adults: a clinical approach from rheumatologists

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Title: Diagnosis and treatment of autoinflammatory diseases in adults: a clinical approach from rheumatologists
Authors: Koga, Tomohiro / Kawakami, Atsushi
Issue Date: 3-Feb-2019
Publisher: Taylor & Francis Ltd.
Citation: Immunological Medicine, 41(4), pp.177-180; 2019
Abstract: utoinflammatory diseases are characterized by recurrent systemic inflammation due to dysfunction of the innate immune system, and they are originally the hereditary recurrent fever syndromes that develop in early childhood. Many cases are thus diagnosed in the pediatric field, but there are many cases that are not diagnosed until adulthood, including adult-onset cases. Accordingly, not only pediatricians but also rheumatologists and primary care physicians play a major role in the diagnosis and treatment of autoinflammatory diseases. Broad non-hereditary autoinflammatory diseases such as adult-onset Still disease and Behcet's disease are also often encountered in routine internal medicine practice. With recent developments in genetic testing, the conditions of patients who have been diagnosed and treated as having fevers of unknown etiology have been increasingly classified as autoinflammatory diseases. Regarding treatment in Japan, biological agents (such as canakinumab) that inhibit IL-1β are now administered to patients with autoinflammatory diseases. In this review, we introduce an approach from rheumatologists based on diagnoses and treatment of ‘classical” autoinflammatory diseases in adults,focusing on familial Mediterranean fever, which has a high frequency in adults.
Keywords: Autoinflammatory diseases / familial Mediterranean fever / IL-1β / inflammasome / MEFV gene
URI: http://hdl.handle.net/10069/38865
DOI: 10.1080/25785826.2018.1524105
Rights: ©2019 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group on behalf of the Japanese Society of Clinical Immunology. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Type: Journal Article
Text Version: publisher
Appears in Collections:Articles in academic journal

Citable URI : http://hdl.handle.net/10069/38865

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