Idiopathic recurrent pericarditis as an autoinlammatory disease

Idiopathic recurrent pericarditis (IRP) is a rare disease, although up to 30% of patients with acute pericarditis can have recurrent pericarditis. IRP is currently regarded as an autoinflammatory condition driven by the disorder of the innate immune system. Polyserositis is a typical feature of the monogenic autoinlammatory diseases, e.g. familial Mediterranean fever. Moreover, variants in the genes responsible for these conditions are also observed in patients with IRP. The role of autoinflammation in the pathogenesis of IRP is also supported by the histological evidence and the efficacy of interleukin (IL)-1 inhibitors, which block the principal mediator of autoinlammation. The authors report the case of IRP that was effectively treated with goflicicept, the new IL-1 inhibitor. Clinical signs and symptoms, diagnosis and treatment of IRP are reviewed.