A case of recurrent pericarditis with SH3BP2 gene variant: a new pathophysiological mechanism?

Dear Editor, Most cases of recurrent pericarditis (RP) are idiopathic and self-limiting, managed outpatient with NSAIDs or colchicine. Recurrence or high-risk features including fever, subacute progression, large pericardial effusions or treatment failure warrant further evaluation. Broadly, evaluation for infectious, autoimmune and neoplastic aetiologies should be considered. Prevalence of these underlying aetiologies will vary with practice setting. Presence of autoantibodies, elevation in pro-inflammatory cytokines, and responsiveness to anti-inflammatory and immunosuppressive treatment suggest underlying immune dysregulation. The precise pathophysiology has not been well described. Broadly, pericardial damage leads to stimulation of both B and T cells in the adaptive immune system as wells as the NLRP3 inflammasome, which upregulates IL-1β in the innate immune system (Fig. 1). This basic model provides the foundation for current treatment strategies. Here we present a case of RP refractory to NSAIDs and colchicine with workup suggestive of autoimmunity due to moderate-titre ANA positivity...