Autoinflammatory syndromes as causes of fever of unknown origin.

The systemic autoinflammatory syndromes often present with recurrent fevers. They have proved exceptionally informative about the innate immune system. Although extremely rare, they are important to recognise, as many can now be completely controlled by long-term drug therapies. Diagnosis relies on clinical suspicion followed by genetic testing. © Royal College of Physicians 2015. All rights reserved.