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Highly Accessed Review

Developments in the scientific and clinical understanding of autoinflammatory disorders

Helen J Lachmann* and Philip N Hawkins

Author Affiliations

National Amyloidosis Centre and Centre for Acute Phase Proteins, Department of Medicine, University College London Medical School, Hampstead Campus, Rowland Hill Street, London NW3 2PF, UK

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Arthritis Research & Therapy 2009, 11:212  doi:10.1186/ar2579

Published: 30 January 2009

Abstract

The autoinflammatory diseases, also known as periodic fever syndromes, are disorders of innate immunity which can be inherited or acquired and which cause recurrent, self-limiting, seemingly spontaneous episodes of systemic inflammation and fever in the absence of autoantibody production or infection. There has been much recent progress in elucidating their aetiologies and treatment. With the exception of familial Mediterranean fever, which is common in certain populations, autoinflammatory diseases are mostly rare but should not be overlooked in the differential diagnosis of recurrent fevers since DNA diagnosis and effective therapies are available for many of them.