• Hal M Hoffman and Anna Simon.
• University of California at San Diego, La Jolla, CA 92093, USA. hahoffman@ucsd.edu
• Nat Rev Rheumatol. 2009 May 1;5(5):249-56.

AbstractRheumatologists are likely to be asked to evaluate patients with recurrent febrile syndromes, so it is important that they are familiar with the clinical and diagnostic features, pathophysiology and therapeutic options for these rare autoinflammatory disorders. These syndromes are all characterized by recurrent episodes of fever and systemic inflammation; however, some syndromes have unique historical and physical features that can help with making a diagnosis. The primary associated morbidity is systemic amyloidosis, usually with renal involvement. Diagnostic testing is mostly limited to genetic testing. NSAIDs, colchicine and corticosteroids have roles in the treatment of some of these disorders, but biologic drugs that target interleukin-1beta are emerging as consistently effective therapies.

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