Periodic fever and autoinflammatory syndromes — viva

Opening (must-hit)

"I hold the periodic fever syndromes on a single framework. They are autoinflammatory, meaning they are innate, antigen-independent and without autoantibodies, driven by the inflammasome and interleukin-one-beta. The five I carry are familial Mediterranean fever, PFAPA, mevalonate kinase deficiency, the tumour necrosis factor receptor-associated periodic syndrome, and the cryopyrin-associated periodic syndromes. My first move in every case is to confirm the clockwork pattern with a fever diary and to exclude the mimics, infection and malignancy, before I label the syndrome. Then I match the drug to the pathway: colchicine for familial Mediterranean fever to prevent the amyloidosis, interleukin-one blockade for the cryopyrin spectrum and mevalonate kinase deficiency, etanercept for the tumour necrosis factor receptor-associated periodic syndrome, and corticosteroid-abort with tonsillectomy for the refractory PFAPA." ^[1][4]