Include rare, treatable IL-1-mediated autoinflammatory diseases in differential diagnosis of chronic or periodic inflammation

Four very rare interleukin (IL)-1-mediated systemic autoinflammatory diseases [cryopyrin-associated periodic syndromes, tumour necrosis factor-associated periodic syndrome, mevalonate kinase deficiency and deficiency of the IL-1 receptor antagonist (DIRA)] can now be definitively diagnosed and effectively treated, potentially transforming patients’ quality of life. First-line therapies are three subcutaneously administered IL-1 blockers: anakinra, rilonacept and canakinumab. Anakinra and rilonacept block IL-1⍺/β and are preferred in DIRA and for neurological or bone symptoms, but canakinumab offers the advantage of monthly or bimonthly injections. Lifelong therapy and monitoring aim to control inflammation, limit consequent organ damage and optimise life quality.