Idiopathic recurrent pericarditis

Idiopathic recurrent pericarditis (IRP) is an inflammatory condition of the pericardium — the thin, two-layered sac surrounding the heart — that recurs after an initial episode of acute pericarditis for which no specific underlying cause (such as infection, autoimmune disease, or malignancy) can be identified. The term 'idiopathic' indicates that the origin remains unknown, though an autoinflammatory or autoimmune mechanism is strongly suspected. Recurrence is defined as a return of pericarditis symptoms after a symptom-free interval of at least 4–6 weeks following the initial episode. The condition primarily affects the cardiovascular system, and patients typically experience sharp or stabbing chest pain that worsens with inspiration or when lying flat, pericardial effusion (fluid accumulation around the heart), and sometimes fever and general malaise. In some cases, a pericardial friction rub can be heard on examination, and electrocardiographic changes or elevated inflammatory markers (such as C-reactive protein) are present. Recurrences can be debilitating and significantly impair quality of life, occurring in approximately 15–30% of patients after a first episode of acute pericarditis and in up to 50% of those who have already experienced one recurrence. The condition can affect individuals of any age but is most commonly diagnosed in adults. Treatment has traditionally relied on nonsteroidal anti-inflammatory drugs (NSAIDs) combined with colchicine, which has been shown to reduce the rate of recurrence. Corticosteroids may be used in refractory cases, though they are associated with an increased risk of further recurrences and side effects. A major advance in treatment has been the approval of rilonacept, an interleukin-1 (IL-1) inhibitor, specifically for recurrent pericarditis in patients who do not respond adequately to conventional therapy. Anakinra, another IL-1 receptor antagonist, has also demonstrated efficacy in clinical trials and is used off-label in some settings. These biologic therapies target the autoinflammatory pathway believed to drive recurrent episodes and represent a significant improvement in the management of this condition.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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