NORD gratefully acknowledges Etienne Leveille, MD Candidate, McGill University School of Medicine and Martin LeWinter, MD, Cardiology Unit, University of Vermont College of Medicine and University of Vermont Medical Center for assistance in the preparation of this report.
Recurrent pericarditis is a disease characterized by recurrent episodes of inflammation of the pericardium, which is the sac containing the heart. The main symptom associated with an episode of pericarditis is chest pain that is typically sharp and worse when taking a deep breath (pleuritic). Shortness of breath (dyspnea) also occurs frequently. Recurrent pericarditis can develop in individuals of any age. The first-line therapy for pericarditis, including for recurrent cases, is a combination of colchicine and either aspirin or non-steroidal anti-inflammatory drugs such as ibuprofen. Although recurrent pericarditis can significantly affect quality of life, it is typically not life threatening or associated with serious illness, and patients are usually well between episodes.
As discussed below, there are numerous causes (etiologies) of pericarditis. Most cases of recurrent pericarditis are idiopathic, that is, the specific cause is not known. Pericarditis is often classified based on the timing of symptoms. A new-onset episode of pericarditis is called acute pericarditis. Episodes lasting more than 4 to 6 weeks but less than 3 months are called incessant pericarditis, while episodes lasting more than 3 months are known as chronic pericarditis. Recurrent pericarditis is defined as an episode of acute pericarditis that occurs at least 4 to 6 weeks after the resolution of a prior episode. Recurrences can occur months or even years after an initial episode.
The main symptom of pericarditis is chest pain, which is present in the vast majority of affected individuals. Typically, the pain is described as sharp and worse when coughing or taking a deep breath (pleuritic). The typical pain seen in pericarditis is also worse when lying down and is partially relieved when leaning forward. It can radiate to the neck, upper back or shoulders. Other symptoms that are associated with pericarditis include shortness of breath (dyspnea), fever, fatigue, malaise and the sensation of an irregular heartbeat (palpitations). Another feature frequently seen in pericarditis is accumulation of fluid in the space between the heart and the pericardium (pericardial sac), which is known as a pericardial effusion. The symptoms seen with a second or subsequent episode of recurrent pericarditis are often similar to the first event, although they tend to be less severe with recurrences. An episode of pericarditis can last days to weeks or longer. Although the symptoms of pericarditis can significantly affect quality of life, affected individuals typically have no symptoms between episodes. The number of episodes of recurrent pericarditis varies greatly between patients.
The two most serious complications of pericarditis are an effusion causing cardiac tamponade and constrictive pericarditis. Cardiac tamponade occurs when a pericardial effusion becomes large enough to impair contraction of the heart. Symptoms of cardiac tamponade include dyspnea, chest discomfort, fatigue, fluid accumulation in the body (edema), and low blood pressure (hypotension). In severe cases, cardiac tamponade can impair cardiac function to the point where it compromises delivery of blood and oxygen to the organs (cardiogenic shock). Constrictive pericarditis is a consequence of chronic pericardial inflammation and is characterized by scarring (fibrosis) and loss of elasticity of the pericardium. The main symptoms of constrictive pericarditis are dyspnea, edema, shortness of breath when lying flat (orthopnea) and chest pain. Fortunately, both cardiac tamponade and constrictive pericarditis are very rare complications of recurrent idiopathic pericarditis, although the risk of constrictive pericarditis is higher with certain other causes of pericarditis (see the “Causes” section below for more details). Overall, most patients can live a productive life with a very low risk of mortality related to recurrent pericarditis.
The causes of pericarditis can be divided in two major categories: those that lead to isolated pericarditis and systemic diseases that can involve the pericardium as one of their manifestations. Most cases of isolated pericarditis are idiopathic. Viral infections have been considered to initiate first episodes of idiopathic pericarditis, but it is not clear how often this is actually the case. Dysfunction of the immune system is thought to play a role in recurrent cases of idiopathic pericarditis. Bacteria (notably tuberculosis), parasites and fungi can also be implicated more rarely, but it is unusual for these non-viral infections to be limited to the pericardium. Pericarditis can also occur after a heart attack (peri-infarction pericarditis and Dressler syndrome) or after cardiac surgery (post-pericardiotomy syndrome) and other types of invasive cardiac procedures. These types of pericarditis are known collectively as post-cardiac injury pericarditis.
Numerous systemic diseases have pericardial involvement as one of their possible manifestations. These include metabolic disturbances such as kidney failure (uremia) and certain medications which cause an immune response affecting the pericardium. Invasion of the pericardium by metastatic cancer (neoplastic pericarditis) is not rare.
Most of systemic disorders that cause pericarditis are characterized by either an immune system that mistakenly attack one’s own body (autoimmune diseases) or by uncontrolled inflammation (autoinflammatory syndromes). Examples of autoimmune diseases associated with pericarditis include systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Behçet’s disease. Autoinflammatory syndromes are rare and usually inherited. The most common in which pericarditis occurs is familial Mediterranean fever (FMF).
The underlying cause of pericarditis is the strongest predictor of the risk of developing constrictive pericarditis, although this complication remains rare overall. The highest risk is seen with bacterial pericarditis, especially tuberculosis, intermediate risk is seen with immune-mediated and neoplastic pericarditis and the risk is low in viral and post-cardiac injury pericarditis. Idiopathic recurrent pericarditis does not seem to confer a significant risk of developing constrictive pericarditis.
Pericarditis is estimated to affect about 28 individuals per 100,000 in the general population every year. Apart from individuals with predisposing conditions, the most frequently affected group are men between the ages of 20 and 50. However, the disease can occur in people of any demographic, including children. It is estimated that about 15 to 30% of individuals that have an initial episode of idiopathic pericarditis will develop recurrent pericarditis.
The diagnostic evaluation of a patient with suspected pericarditis begins with a complete patient history and physical examination in order to evaluate risk factors, signs and symptoms of the disease, and features that could suggest an alternative diagnosis. An essential part of the physical examination is auscultation of the heart using a stethoscope; in some patients, a characteristic scratching sound, known as a pericardial friction rub, may be heard. The physical exam can also show signs of cardiac tamponade or constrictive pericarditis, such as dyspnea, distended neck veins, edema, or low blood pressure.
After gathering a complete history and performing an appropriate physical examination, certain tests will be performed in all patients with suspected pericarditis. An electrocardiogram, which measures electrical activity of the heart, might show characteristic changes associated with pericarditis and can help rule out other cardiac causes of chest pain. Blood levels of troponin, a protein that is released into the blood following damage to cardiac muscle, are also useful to differentiate pericarditis from other heart conditions. Troponin levels are usually normal in isolated pericarditis but are elevated in myopericarditis, myocarditis and myocardial infarction. Other routine laboratory tests include a complete blood count, which can show an increase in white blood cells due to inflammation, and certain inflammatory markers, namely, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Routine imaging tests include a chest X-ray, which is usually normal in pericarditis but can show signs of an alternative diagnosis or a pericardial effusion if it is large. An echocardiogram, an imaging method that uses sound waves to evaluate the anatomy and function of the heart and pericardium, is also routinely performed in patients with suspected pericarditis.
Certain tests are only performed in a subset of patients. For instance, if the diagnosis of pericarditis is not confirmed with routine tests, advanced imaging studies can be performed, notably computed tomography (CT) or magnetic resonance imaging (MRI) of the heart. If a bacterial or neoplastic pericarditis is suspected and a significant pericardial effusion is present, pericardial fluid can be removed for analysis. This is done during a procedure known as pericardiocentesis, where a needle is inserted into the pericardial space to drain the effusion. Very rarely, a sample of pericardial tissue can be harvested for analysis (pericardial biopsy). Additional laboratory tests that might be performed depending on the clinical scenario include blood cultures if bacterial pericarditis is suspected, specific tests (e.g., antinuclear antibody levels) if an autoimmune disease is suspected or a tuberculin skin test if tuberculosis is suspected. The findings of routine or more advanced tests may also determine the need for additional testing if necessary.
In every patient with pericarditis, restriction of physical activity is recommended until symptoms have resolved and inflammatory markers have normalized. If a systemic disease is identified as the cause of pericarditis, therapy should be focused on treating the underlying condition. For instance, antibiotics will be required for a patient with tuberculosis, and chemotherapy or other treatments will be required in a patient with neoplastic pericarditis. Another important consideration is whether the affected individual needs to be admitted to the hospital or can be treated as an outpatient. Although most patients can be treated outside the hospital, patients with high-risk features are usually admitted. These features include fever, a slow (subacute) onset of disease without sudden onset of chest pain, presence of a large pericardial effusion, use of immunosuppressant medications or blood thinners (anticoagulants) or high troponin levels (which suggests myopericarditis).
Patients with viral or idiopathic pericarditis are treated with a combination of colchicine and either aspirin or other non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen, naproxen or indomethacin. This medication combination is also the first line of treatment in most patients with recurrent pericarditis. These medications are continued at least until symptoms have resolved and inflammatory markers have normalized. In patients with contraindications to NSAIDs or no improvement with first-line therapy, NSAIDs can be replaced with prednisone, a corticosteroid drug with powerful anti-inflammatory properties. Patients who still have symptoms despite treatment with colchicine and prednisone may benefit from addition or continuation of a NSAID. In refractory cases, other therapies may be used, although their efficacy has yet to be confirmed in controlled clinical trials. These therapies inhibit the immune system to decrease inflammation and include azathioprine, methotrexate and intravenous immune globulins (IVIG). None of these have been tested in controlled clinical trials.
Most recently, drugs that block the effects of a specific immune pathway known as interleukin-1 (IL-1) have been used with great success in patients with refractory, recurrent idiopathic and post-cardiac injury pericarditis. These drugs are so-called “biologics”, which are proteins that selectively block this pathway and must be injected under the skin (subcutaneously). The two IL-1 antagonists that have been employed to date are anakinra and rilonacept.
In 2021, Arcalyst (rilonacept) was approved by the U.S. Food and Drug Administration (FDA) to treat recurrent pericarditis and reduce the risk of recurrence in adults and children 12 years and older.
As a last resort, surgery where the pericardium is removed can be performed (pericardiotomy). Pericardiotomy might also be performed if constrictive pericarditis has developed. In the rare cases where cardiac tamponade develops, pericardiocentesis or surgical drainage of the pericardial effusion might have to be performed.
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