[Fever of unknown cause and autoinflammatory disease]

Autoinflammatory diseases are often associated with various kinds of febrile episodes such as fever of unknown origin, periodic fever and recurrent fever. Therefore, in the differential diagnosis of fever of unknown cause, autoinflammatory diseases should be considered after exclusion of infections, malignancy and autoimmune diseases. As autoinflammatory diseases now include TRAPS (TNF receptor-associated periodic syndrome), CAPS (cryopyrin -associated periodic syndromes), FMF(familial Mediterranean fever), MAPS (mevalonate kinase-associated periodic fever syndrome, hyper-IgD syndrome) and many others, and show symptoms and signs of wide variations, we need to make an accurate diagnosis of them to prevent possible complications such as amyloidosis.