Auto-inflammatory diseases are a rapidly moving field, where the description of new entities, the understanding of the pathogenesis and the treatment has shown important progress in the past years. This group of conditions include monogenic auto-inflammatory diseases, like FMF, CAPS, TRAPS and MKD, and non-monogenic autoinflammatory diseases, like PFAPA, SoJIA, CRMO, and Behçet. The most common...

Autoinflammatory diseases are defined as recurrent attacks of systemic inflammation that are often unprovoked (or triggered by a minor event) related to a lack of adequate regulation of the innate immune system. Within the past decade, the list of autoinflammatory diseases has included cryopyrin-associated periodic syndromes, familial Mediterranean fever, mevalonate kinase deficiency, tumor nec...