Recurrent pericarditis involves repeated episodes of inflammation affecting the pericardium, the two-layered sac surrounding the heart. This sac normally contains a small amount of fluid, acting as a lubricant to reduce friction as the heart beats. When inflamed, the pericardium can cause symptoms like sharp chest pain, often worsened by deep breaths or lying flat.
The primary goals of treating recurrent pericarditis are to alleviate these uncomfortable symptoms, reduce the inflammation, and prevent future episodes. Achieving these aims typically involves a tailored approach, often combining various medical therapies to manage the condition effectively.
Foundational Medical Therapies
The initial approach to managing recurrent pericarditis involves a combination of nonsteroidal anti-inflammatory drugs (NSAIDs) and colchicine. This dual therapy is the standard first-line treatment. NSAIDs, such as ibuprofen or high-dose aspirin, work by inhibiting enzymes involved in producing prostaglandins, which are substances that contribute to inflammation and pain. This action helps lessen the chest pain and reduce the inflammatory response within the pericardial sac.
Alongside NSAIDs, colchicine is added to the treatment regimen to prevent the inflammation from returning. Colchicine is thought to interfere with the functions of certain white blood cells and inhibit microtubule formation, thereby reducing inflammation. Studies have shown that adding colchicine can significantly lower the risk of future recurrences, sometimes halving the incidence of subsequent flares. This combination therapy is usually continued for several months, often for six to twelve months, to ensure sustained suppression and minimize relapse.
The Role of Corticosteroids
Corticosteroids, such as prednisone, are potent anti-inflammatory medications that can effectively reduce pericardial inflammation and alleviate symptoms. However, they are not considered a primary treatment choice for recurrent pericarditis. Their use is reserved for individuals who cannot tolerate or do not respond adequately to NSAIDs and colchicine, or for those with specific underlying conditions, such as autoimmune diseases or pregnancy.
Corticosteroid use in recurrent pericarditis carries an increased risk of recurrence, often referred to as “steroid-dependency.” While they can offer quick symptom relief, long-term or high-dose steroid therapy has been linked to a higher chance of future inflammatory episodes. Higher doses are associated with more side effects, recurrences, and hospitalizations. Therefore, if corticosteroids are necessary, they are typically used at the lowest effective dose for the shortest duration, with a gradual tapering schedule to reduce the risk of rebound inflammation.
Targeted Immunotherapies
For patients whose recurrent pericarditis does not respond to foundational medical therapies or who become dependent on corticosteroids, targeted immunotherapies are an advanced treatment option. These therapies focus on specific components of the immune system that drive the inflammatory process. Research has identified Interleukin-1 (IL-1) as a key protein involved in the inflammation seen in many cases of recurrent pericarditis.
IL-1 inhibitors are designed to block the activity of this pro-inflammatory cytokine. Rilonacept, an FDA-approved IL-1 inhibitor for recurrent pericarditis, acts as a “decoy receptor” that binds to circulating IL-1 alpha and IL-1 beta proteins. By trapping IL-1, rilonacept prevents these inflammatory signals from reaching their cell surface receptors, interrupting the inflammatory cascade. This targeted approach aims to address the underlying immune dysregulation, offering a promising option for recurrent pericarditis.
Surgical Intervention
Surgical intervention is a last-resort treatment for recurrent pericarditis, reserved for patients with severe, persistent, and debilitating symptoms that have not responded to medical management. The primary surgical procedure is a pericardiectomy, which involves removing part or all of the pericardium, the sac surrounding the heart. This major cardiac surgery aims to permanently eliminate the inflamed or scarred tissue causing recurrent inflammation and restricting heart function.
Pericardiectomy is most commonly performed for constrictive pericarditis, a complication of chronic inflammation where the pericardium becomes stiff and thick, hindering the heart’s ability to fill properly. Removing the constricting pericardium allows the heart to move and expand more freely, restoring normal blood flow and alleviating symptoms of heart failure. While it can offer relief and potentially a cure for constriction, it is a complex procedure with associated risks, including bleeding, abnormal heart rhythms, and nerve injury. Despite being a major operation, it offers an option for otherwise unmanageable recurrent pericarditis.