Recurrent
pericarditis (RP) is a troublesome and debilitating complication of acute
pericarditis. Although the etiopathogenesis of this condition remains unknown, an intricate overlap of autoimmune and autoinflammatory pathways has been hypothesized to explain its beginning and recurrence over time. The majority of cases are defined as "idiopathic", reflecting our awkwardness to unravel the intimate mechanisms of RP. Given the possible occurrence of anti-nuclear, anti-heart and anti-intercalated disk
antibodies as well as the association with peculiar
human leukocyte antigen haplotypes, an autoimmune contribution has been claimed to specify the nature of RP. However, the most innovative pathogenic scenario of RP has been conferred to the innate immune system, mainly involving neutrophils and macrophages that produce a large amount of
interleukin (IL)-1 via
inflammasome activation. The clinical resemblance of RP with autoinflammatory diseases that may be marked by symptomatic
serositis, high
fevers and strikingly increased inflammatory parameters further suggests a similar
inflammasome-mediated pathogenesis.
Aspirin or non-steroidal anti-inflammatory drugs (
NSAIDs) remain the mainstay of
therapy in RP, whereas
colchicine is recommended on top of standard anti-inflammatory
therapy, due to its role in inhibiting the
IL-1 converting
enzyme (
caspase 1) within the
inflammasome as well as the release of additional pro-inflammatory mediators and
reactive oxygen species. With regard to treatment of RP refractory to
NSAIDs and
colchicine, blockade of
IL-1 is the most relevant advance achieved in the last decade: the outstanding effect of the short-acting
IL-1 receptor antagonist
anakinra has been first recognized in the pediatric population, giving a proof of its practical feasibility. Over a more recent time, a growing experience with
anakinra deriving from both large and small studies has further confirmed that RP might be regarded as an IL-1-mediated disease. This review aims to provide a contemporary insight into the mechanisms leading to RP as well as into the most recent literature data showing the beneficial approach originating from
IL-1 blockade in this intriguing disorder.