Periodic fever syndromes

Periodic fever syndromes are rare disorders of the immune system characterized by episodes of systemic inflammation. Although there are similarities in the presentation, most of these disorders have a distinct pathophysiology and clinical features, with the duration of attacks aiding diagnosis. The precise classification can remain difficult in some cases. Recent advances in the understanding of the pathogenesis of periodic fever syndromes have led to the development of more effective therapies. Diagnosis requires a high degree of clinical suspicion, appropriate laboratory and molecular testing as well as investigations to exclude other causes. Diagnosis allows the use of targeted therapies and the avoidance of complications such as systemic amyloidosis.