Autoinflammatory diseases: cryopyrin-associated periodic syndromes presenting in the newborn and first year of life

Autoinflammatory diseases are disorders of the innate immune system, characterised by recurrent attacks of fever and systemic inflammation in the absence of infection. This article focuses on a group of hereditary autoinflammatory conditions (or periodic fever syndromes), the cryopyrin-associated periodic syndromes (CAPS) that can have a neonatal onset, particularly chronic infantile, neurological, cutaneous and articular (CINCA) syndrome. The clinical presentation, diagnostic investigations, treatment and outcome of CAPS are reviewed.