Pericarditis

History

• Sudden onset of retrosternal pleuritic chest pain is most common presenting symptom ^r7c1
  • Typically sharp and pleuritic ^c2c3
  • Often exacerbated by deep inspiration, coughing, or lying flat and relieved by sitting up and/or leaning forward ^c4
  • May radiate to the neck, arms, left shoulder, or 1 or both trapezius muscle ridges ^c5c6c7
  • Uncommon presentation in purulent pericarditis
• Other symptoms and onset are influenced by underlying cause and disease course: ^r7
  • Fever (more common in children than adults) ^r1c8
    • Fevers due to bacterial pericarditis occur at regular intervals and are associated with frank rigors ^r7
  • Malaise and myalgia may be present ^r8c9c10
  • If pericardial effusion develops, dyspnea, dry cough, dysphagia, hiccups, and dysphonia may result ^{r9c11c12c13c14}
  • Symptoms of heart failure suggest progression to constrictive pericarditis ^r1
• History may suggest an underlying cause of suspected pericarditis
  • Patients with viral or bacterial pericarditis may have a history of recent viral or bacterial illness, respectively ^r1
  • Patients may have known underlying autoimmune disease or history of lung or breast cancer, malignant melanoma, lymphoma, or leukemia ^r1
  • Bacterial pericarditis is always an acute disease
  • Fungal pericarditis evolves more slowly
  • Onset of tuberculous pericarditis is insidious

Physical examination

• Patient is typically normotensive ^r8c15
• Sinus tachycardia may be evident and associated with fever ^c16
• Patient may be febrile if cause is infectious ^c17
• Clinical signs of sepsis (eg, fever, hypotension) may accompany purulent pericarditis ^r7c18
• Pericardial friction rub may be audible on auscultation over the left sternal border (35% of patients) ^r7c19
  • Pericardial rub is triphasic (early diastole, late diastole, and systole) in 50% of patients, biphasic in 33% of patients, and monophasic in 13% of patients ^r3
  • Typically high-pitched, scratchy, or squeaky and may vary in intensity over time
  • Often varies throughout the day; it is important to evaluate patient multiple times and in different positions ^r2
• Clinical signs that acute pericarditis has progressed to constrictive pericarditis include: ^r1
  • Elevated jugular venous pressure ^r10c20
    • Kussmaul sign (elevation of jugular venous pressure on inspiration) ^c21
      • May not be visible in milder forms of constrictive pericarditis
      • When visible, it can be seen at the upper neck or angle of the jaw when the patient is sitting; extremely high venous pressure may not be visible unless the patient is standing
  • Altered venous waveforms: a brisk collapse of the neck veins may be apparent, representing prominent x and y descents ^r10
  • Pericardial knock on auscultation; may be difficult to distinguish from an S₃ ^r7c22
  • Hyperdynamic apical impulse identified on palpation ^r10c23
  • Signs of right-sided heart failure: leg edema, abdominal distention, hepatomegaly, and ascites ^{r10c24c25c26c27}
• Pulsus paradoxus and Kussmaul sign with elevated jugular venous pressure suggest disease is complicated by cardiac tamponade; other signs include: ^r7
  • Hypotension ^c28
  • Tachycardia ^c29
  • Muffled heart sounds ^c30

Causes

• Pericarditis may be categorized by infectious or noninfectious causes ^r1
  • Infectious causes
    • Viral (most common)
      • Majority of idiopathic cases of pericarditis are presumed to be viral; causes include:
        • Enteroviruses ^c31
        • Herpesviruses ^c32
        • Adenoviruses ^c33
        • Human parvovirus B19 ^c34
    • Bacterial
      • Mycobacterium tuberculosis is most common bacterial cause ^c35
        • Tuberculous pericarditis accounts for 4% or less of pericardial disease in developed countries but accounts for a significant portion of pericardial disease (50%-90%) in tuberculosis-endemic countries ^r1
      • Other bacteria include Coxiella burnetii and Borrelia burgdorferi^c36c37
      • Rarely, caused by:
        • Pneumococcus ^c38
        • Meningococcus ^c39
        • Gonococcus ^c40
        • Providencia stuartii^c41
        • Streptococcus species ^c42
        • Staphylococcus species ^c43
        • Haemophilus species ^c44
        • Chlamydia species ^c45
        • Mycoplasma species ^c46
        • Legionella species ^c47
        • Leptospira species ^c48
        • Listeria species ^c49
    • Fungal (rare)
      • Infections with Histoplasma species are more likely in immunocompetent patients ^c50
      • Infections with Aspergillus, Blastomyces, and Candida species are more likely in immunocompromised patients ^c51c52c53
    • Parasitic causes are very rare but include infection with species of the genera Echinococcus and Toxoplasma ^c54c55
  • Noninfectious causes
    • Autoimmune (common)
      • Systemic autoimmune or inflammatory conditions
        • Systemic lupus erythematosus ^c56
        • Sjögren syndrome ^c57
        • Rheumatoid arthritis ^c58
        • Scleroderma ^c59
      • Systemic vasculitides
        • Takayasu disease ^c60
        • Eosinophilic granulomatosis with polyangiitis ^c61
      • Sarcoidosis ^c62
      • Familial Mediterranean fever ^c63
      • Inflammatory bowel disease ^c64
      • Still disease ^c65
    • Neoplastic
      • Typically caused by secondary metastatic tumors; lung cancer, breast cancer, and lymphoma are most common ^c66
      • Rarely, caused by primary tumors (primarily pericardial mesothelioma) ^c67
    • Metabolic
      • Uremia ^c68
      • Myxedema ^c69
      • Anorexia nervosa ^c70
    • Traumatic
      • Early onset (rare)
        • Direct injury (eg, penetrating thoracic injury, esophageal perforation) ^c71c72
        • Indirect injury (eg, nonpenetrating thoracic injury, radiation injury) ^c73c74
          • Most radiation injury cases are secondary to radiation for Hodgkin lymphoma or breast or lung cancer
      • Delayed onset (common) ^c75
        • Post–myocardial infarction syndrome
          • Dressler syndrome: early infarct-associated (postinfarction) pericarditis
            • Typically occurs a few days after acute myocardial infarction
            • Rare and often transient
          • Late pericarditis (postcardiac injury): typically occurs 1 to 2 weeks after acute myocardial infarction
        • Postpericardiotomy syndrome ^c76
        • Posttraumatic (may include those occurring after iatrogenic trauma) ^c77
        • Late onset of pericardial disease is also common after radiation (20% within 2 years) and typically consists of effusive-constrictive pericarditis or classic constrictive pericarditis ^c78
    • Drug related (rare)
      • Drug-induced lupus syndrome secondary to use of procainamide, hydralazine, methyldopa, and phenytoin ^c79c80c81c82
      • Chemotherapy agents including doxorubicin, daunorubicin, cyclophosphamide, and 5-fluorouracil ^c83c84c85c86
      • Other drugs include penicillins, amiodarone, methysergide, mesalazine, clozapine, minoxidil, dantrolene, phenylbutazone, thiazides, streptomycin, thiouracils, streptokinase, p-aminosalicylic acid, sulfa drugs, cyclosporine, bromocriptine, granulocyte-macrophage colony-stimulating factor, and anti–tumor necrosis factor agents ^{c87c88c89c90c91c92c93c94c95c96c97c98c99c100c101c102c103c104}
    • Other
      • Amyloidosis ^c105
      • Aortic dissection ^c106
      • Pulmonary arterial hypertension ^c107
      • Chronic heart failure ^c108

Risk factors and/or associations

Primary diagnostic tools

• Suspect pericarditis in patients presenting with pleuritic chest pain, particularly if it is improved by sitting upright or leaning forward, and a pericardial friction rub
• Initial workup for all patients with suspected pericarditis includes the following: ^r1
  • ECG ^c124
  • Chest radiography ^r3c125
  • Laboratory studies ^r2
    • CBC with differential ^c126
    • C-reactive protein and/or erythrocyte sedimentation rate ^c127c128
    • Cardiac troponin level ^c129
    • Renal function and liver tests ^c130c131
  • Transthoracic echocardiography ^c132
• Additional diagnostic testing is individualized and may include: ^r1
  • Blood cultures in patients with suspected purulent pericarditis (eg, fever, signs of sepsis, concomitant bacterial infection) ^c133
  • Cardiac CT or MRI in patients with suspected constrictive pericarditis, nondiagnostic echocardiography, or complicated course ^c134c135
  • Tuberculosis testing (eg, interferon-γ release assay; culture of sputum, gastric aspirate, and/or urine for Mycobacterium tuberculosis) and chest CT in patients suspected of having tuberculous pericarditis (eg, those who reside in tuberculosis-endemic areas, immunocompromised patients) ^c136
  • HIV serology ^c137
  • Antinuclear antibodies in patients with suspected rheumatologic disorders
    • Additional tests may include antineutrophil cytoplasmic autoantibody, extractable nuclear antigens, ferritin level if Still disease is suspected, and PET if large vessel arteritis or sarcoidosis is suspected ^c138c139c140
  • Consider specific neoplastic markers and CT of chest and abdomen for patients with suspected neoplastic pericarditis ^c141c142
  • Pericardiocentesis for patients with suspected bacterial, neoplastic, or refractory pericarditis; perform therapeutically in patients with cardiac tamponade ^c143
  • Pericardial biopsy in patients suspected of having neoplastic pericarditis or in those suspected of having tuberculous pericarditis with more than 3 weeks of symptoms and no cause diagnosed by other methods ^c144
  • Cardiac catheterization can be used to diagnose constrictive pericarditis when other diagnostic methods are inconclusive ^r1c145
• Although it is not necessary to identify the underlying cause for pericarditis in all patients, it is important to rule out causes that require targeted therapy; this may require additional testing ^r1
  • Pericarditis secondary to malignancy
  • Pericarditis associated with autoimmune disease
  • Tuberculous pericarditis
• For patients with recurrent idiopathic pericarditis and symptom-free periods between episodes, it is unnecessary to perform a new search of disease cause with each recurrence unless new symptoms present ^r1
• For patients with suspected constrictive pericarditis, also obtain B-type natriuretic peptide and evaluate for tuberculosis ^r1

Laboratory

• CBC ^c146
  • Characterized by leukocytosis ^r1
• Inflammatory markers
  • Characteristically associated with elevated erythrocyte sedimentation rate ^c147
  • C-reactive protein levels are elevated (greater than 3 mg/L) in 75% of cases; however, some patients may present with C-reactive protein levels within reference range that increase within the first week of disease ^r11c148
• Cardiac troponin levels
  • Troponin I and T levels are elevated in 35% to 50% of acute pericarditis cases as a result of epicardial inflammation ^r9c149c150
  • Correlates with ST-segment elevation on ECG ^r9
  • Typically return to reference-range levels within 1 to 2 weeks
    • Elevated troponin levels lasting longer than 2 weeks suggest myocardial involvement and indicate worse prognosis ^r9

Imaging

• Chest radiography ^r1c151
  • Recommended as an initial imaging technique for all suspected cases of acute or recurrent pericarditis; may detect mild to moderate pericardial effusions and identify potential causes (eg, tuberculosis, pneumonia, neoplasm)
  • May show normal findings in patients with acute pericarditis or may show pericardial effusion
  • Mediastinal irregularities indicate lymphadenopathy from tuberculous or fungal pericarditis ^r9
  • May show pericardial calcifications suggestive of constrictive pericarditis ^r1
• Transthoracic echocardiography ^r1c152
  • Indicated as first line imaging modality for patients with suspected pericarditis; it is a primary method to detect pericardial effusion ^r1
  • Critical for evaluating hemodynamic effects on the heart if disease is suspected to be complicated by cardiac tamponade or progression to constrictive pericarditis ^r2
  • Able to identify disease complicated by myocardial involvement ^r3
  • The following findings are typical of acute or recurrent pericarditis:
    • Patients may have normal echocardiography findings ^r1
    • Pericardial effusion may be evident ^r1
      • Size of pericardial effusion can be assessed by determining end-diastolic distance of echo-free space between the epicardium and parietal pericardium on 2-dimensional echocardiography
        • Less than 10 mm: small
        • 10 to 20 mm: moderate
        • Greater than 20 mm: large
      • A large pericardial effusion with frondlike projections and thick, porridgelike fluid is suggestive of, but not specific for, tuberculous pericarditis
    • Thickened or hyperreflective pericardial layers (normal pericardial thickness is 0.7-2 mm) ^r1
    • Wall motion abnormalities may indicate myocardial involvement (myopericarditis) ^r1
    • Intrapericardial fibrinous strands may be evident ^r1
  • The following findings are consistent with constrictive pericarditis:
    • Septal motion abnormalities ^r10
    • Pericardial thickening or calcifications
    • Dilated atria ^r1
    • Dilation and diminished collapse of inferior vena cava is a universal finding in constrictive pericarditis ^r1
    • Premature opening of the pulmonary valve ^r1
    • Restrictive filling pattern of the right and left ventricles, suggesting stiff pericardium ^r1
    • Respiratory variation of the mitral peak E-wave velocity of greater than 25% on mitral inflow pulsed-wave Doppler echocardiography; 33% of patients may not present with this variation ^r10
    • Normal or increased mitral annular velocity (greater than 7 cm/second) ^r1
    • Decreased expiratory diastolic hepatic vein velocities with large reversals (highly specific for constrictive pericarditis) ^r10
    • Respiratory variation in the pulmonary venous peak D-wave velocity greater than 20% ^r1
    • Flow propagation velocity greater than 45 cm/second ^r1
    • Greater than 25% fall in mitral inflow velocity and greater than 40% rise in tricuspid velocity in the first beat after inspiration, with opposite changes observed after expiration ^r1
    • Normal or increased propagation velocity of early diastolic transmitral flow using color M-mode echocardiography ^r1
    • Annulus reversus (mitral lateral e′ velocity less than medial e′ velocity) ^r10
• CT ^r1c153
  • Indicated as a second line imaging technique ^r1
    • Indicated to assess calcifications, pericardial thickness, and extent of pericardial involvement in patients with suspected constrictive pericarditis
    • Most accurate method to detect pericardial calcifications
  • The following findings are indicative of acute and recurrent pericarditis:
    • Evidence of pericardial inflammation (eg, edema, pericardial contrast enhancement) ^r1
    • Pericardial thickness greater than 3 to 4 mm ^r1
    • Findings may be more heterogeneous in recurrent pericarditis owing to fibrotic adhesions ^r1
    • Irregular pericardial delineation may present with recurrent disease ^r1
    • Typical pericarditis signs with mediastinal and tracheobronchial lymphadenopathy (greater than 10 mm with hypodense centers and matting visible) with sparing of hilar lymph nodes is suggestive of tuberculous pericarditis ^r1
  • The following findings are indicative of constrictive pericarditis:
    • Mild to moderate thickening of pericardial layers with or without calcifications^r1; 20% of patients with constrictive pericarditis may have normal pericardial thickness^r10
      • Usually most noticeable at the base of the ventricles, atrioventricular grooves, and atria
      • CT is the most accurate imaging modality to evaluate calcifications ^r1
    • Adjacent myocardium may be fibrotic and calcified ^r1
    • Compressed cardiac contents ^r1
    • Abnormally shaped ventricular septum ^r1
    • Dilated atria and caval/hepatic veins ^r1
    • Contrast reversal in caval/hepatic veins ^r1
    • Hepatic congestion ^r1
• Cardiac MRI with gadolinium contrast material ^c154
  • Indicated as a second line imaging modality:
    • To diagnose atypical presentations of constrictive pericarditis, such as cases of transient or effusive-constrictive pericarditis or those with minimally thickened pericardium ^r1
    • To evaluate myocardial involvement and rule out ischemic myocardial necrosis in patients with pericarditis suspected to be complicated by myocardial disease ^r1
  • May identify fibrotic fusion of pericardial layers more easily than CT; however, pericardial calcifications are not visualized ^r1
  • The following findings are indicative of acute or recurrent pericarditis: ^r1
    • Strong pericardial late gadolinium enhancement after contrast ^r1
    • Inspiratory septal flattening on real-time cine cardiovascular MRI due to decreased pericardial compliance ^r1
    • Subepicardial/midwall myocardial late gadolinium enhancement indicates myopericarditis ^r1
    • Findings may be more heterogeneous in recurrent pericarditis owing to fibrotic adhesions ^r1
    • Irregular pericardial delineation may present with recurrent disease ^r1
  • The following findings are indicative of constrictive pericarditis:
    • Ventricular interdependence
    • Fibrocalcific process extending to the myocardium
    • Compressed cardiac contents
    • Dilated atria and caval/hepatic veins
    • Pleural fluid
    • Increased ventricular coupling as seen using real-time cine cardiac MRI or real-time phase contrast imaging
    • Fibrotic adhesion of pericardial layers

Functional testing

• ECG ^c155
  • Recommended as part of initial workup of all patients with suspected acute or recurrent pericarditis; ECG changes are a criterion for diagnosis
    • However, pericarditis does not always cause ECG changes or may cause atypical ECG abnormalities
  • 4 stages of ECG abnormalities; patient may present with any of these findings, depending on timing of examination relevant to onset of disease ^r7
    • Stage 1: widespread ST-segment elevation (typically concave upward) with PR-segment depression suggests inflammation of the epicardium and is a hallmark sign of an acute episode of pericarditis (80% of patients with pericarditis) ^r1
      • Accompanied by reciprocal ST-segment depression in the aVR and V₁ leads ^r7
      • Typically seen within hours to days of chest pain onset ^r7
    • Stage 2: ST and PR segments normalize ^r7
      • Typically occurs within the first week of illness ^r2
    • Stage 3: symmetrical, widespread T-wave inversions ^r7
    • Stage 4: findings may appear normal or T-wave inversions persist indefinitely ^r7
  • Low-voltage, nonspecific ST/T changes, and atrial fibrillation are characteristic of constrictive pericarditis ^r1

Procedures

Other diagnostic tools

• Acute pericarditis diagnosis requires that at least 2 of the following criteria be met: ^r1
  • Chest pain
    • Typically sharp and pleuritic
    • May be improved by sitting up and leaning forward
  • Pericardial friction rub
  • ECG changes
    • Typically new widespread ST elevation or PR depression
  • Pericardial effusion
• Additional supporting findings include:
  • Elevated inflammatory markers
  • Evidence of pericardial inflammation on imaging

Most common

• Myocardial infarction or ischemia ^c158c159d1
  • Presentation is often similar (ie, with chest pain)
  • Unlike pericarditis, chest pain is described as pressurelike, heavy, and squeezing and may resolve with administration of nitroglycerin
    • Pain does not vary with respiration or position changes and lasts minutes to hours rather than hours to days
  • Differentiated based on clinical features, ECG, cardiac biomarker assay, and echocardiography
• Pneumonia ^c160d2
  • Presentation includes fever and productive cough; chest pain and dyspnea may occur
  • Differentiated based on clinical findings and chest radiograph
• Pulmonary embolism ^c161d3
  • Chest pain has similar character but can be anterior, posterior, or lateral in location
  • Chest pain in phase with respiration (no chest pain when patient ceases breathing) and is not positional in nature
  • Differentiated based on clinical features, cardiac enzymes, ECG, and imaging findings; multidetector-row CT angiography is diagnostic for pulmonary embolism
• Pneumothorax ^c162
  • Presentation includes acute dyspnea and pleuritic chest pain
  • Unlike pericarditis, may not be positional in nature and pericardial friction rub is absent
  • Differentiated based on clinical findings and chest radiograph
• Pleuritis (pleurisy) ^c163
  • Often presents with pleuritic inspiratory chest pain, cough, and sometimes dyspnea
  • Unlike pericarditis, occurs in the setting of respiratory infection
  • Differentiated based on clinical features and presence of normal ECG and imaging tests
• Costochondritis ^c164
  • Presents with pleuritic chest pain that may be exacerbated by movement
  • Unlike pericarditis, there is reproducible tenderness with palpation of the costochondral junctions
  • Patient is otherwise well with normal physical examination findings
  • Differentiated based on clinical features and presence of normal ECG and imaging findings

Admission criteria

Patients who present with confirmed or suspected pericarditis and at least 1 of the following risk factors for poor prognosis should be admitted to hospital for diagnosis and determination of cause: ^r1

• Fever higher than 38 °C
• Subacute disease course (symptoms developing over several days without clear-cut onset)
• Large pericardial effusion
• Cardiac tamponade
• Failure to respond to NSAID therapy within 7 days
• Disease in the context of immunosuppression or acute trauma
• Currently taking oral anticoagulant therapy
• Elevated cardiac troponin level (suggests myocarditis)

All other patients can be treated with outpatient care ^r1

Recommendations for specialist referral

• Patients with suspected pericarditis should be evaluated and managed in consultation with a cardiologist
• Refer patients with infectious pericarditis to an infectious disease specialist ^r1
• Refer to interventional cardiologist for management of moderate to large pericardial effusion or cardiac tamponade
• Refer to cardiothoracic surgeon if pericardiotomy or pericardiectomy is required (eg, in constrictive pericarditis) ^r1

Drug therapy

• NSAIDs ^c165c166c167
  • Aspirin ^r1
    • Acute
      • Aspirin Oral tablet; Adults: 750 to 1,000 mg PO every 8 hours for 1 to 2 weeks, then decrease dose by 250 to 500 mg/day every 1 to 2 weeks in combination with colchicine.
    • Recurrent
      • Aspirin Oral tablet; Adults: 500 to 1,000 mg PO every 6 to 8 hours for at least 2 to 4 weeks, then decrease dose by 250 to 500 mg/day every 1 to 2 weeks in combination with colchicine.  Dose range: 1.5 to 4 g/day.
  • Ibuprofen ^r1
    • Acute
      • Treatment duration is dependent on resolution of symptoms and the normalization of markers of inflammation (eg, C-reactive protein).
      • Ibuprofen Oral tablet; Adults: 600 mg PO every 8 hours for 1 to 2 weeks, then decrease dose by 200 to 400 mg/day every 1 to 2 weeks in combination with colchicine.
    • Recurrent
      • Ibuprofen Oral tablet; Adults: 600 mg PO every 8 hours for at least 2 to 4 weeks, then decrease dose by 200 to 400 mg/day every 1 to 2 weeks in combination with colchicine. Dose range: 1,200 to 2,400 mg/day.
  • Indomethacin
    • Acute
      • Indomethacin Oral capsule; Adults: 25 mg PO every 8 hours, initially; increase dose to 50 mg PO every 8 hours as tolerated and continue for 1 to 2 weeks, then decrease dose by 25 mg/day every 1 to 2 weeks in combination with colchicine.
    • Recurrent
      • Indomethacin Oral capsule; Adults: 25 mg PO every 8 hours, initially; increase dose to 50 mg PO every 8 hours as tolerated and continue for at least 2 to 4 weeks, then decrease dose by 25 mg/day every 1 to 2 weeks in combination with colchicine.
• Colchicine ^r1c168
  • NOTE: The 0.5-mg tablet of colchicine is currently not available in the United States and many other countries. The 0.6-mg tablet or dose is used in place of the guideline-recommended 0.5-mg dosing.
    • Colchicine Oral tablet; Adults: 0.5 mg or 0.6 mg PO once daily for patients weighing less than 70 kg.
    • Colchicine Oral tablet; Adults: 0.5 mg or 0.6 mg PO twice daily for patients weighing 70 kg or more.
  • Use for 3 months for acute pericarditis, 6 months for recurrent pericarditis
  • After obtaining a complete response, may be tapered gradually over several weeks to several months; however, this is not mandatory ^r1
• Corticosteroids
  • Prednisone ^r1c169
    • Prednisone Oral tablet; Adults: 0.25 to 0.5 mg/kg PO once daily. Corticosteroids are contraindicated in pericarditis after myocardial infarction; corticosteroids slow myocardial scar formation and incidence of rupture may increase
      • Maintain original dose until symptoms resolve and C-reactive protein levels normalize, then slowly taper.
        • Suggested taper:
          • For daily doses greater than 50 mg, taper by 10 mg/day every 1 to 2 weeks
          • For daily doses 25 to 50 mg, taper by 5 to 10 mg/day every 1 to 2 weeks
          • For daily doses 15 to 24 mg, taper by 2.5 mg/day every 2 to 4 weeks
          • For daily doses less than 15 mg, taper by 1.25 to 2.5 mg/day every 2 to 6 weeks
        • Only decrease dose if the patient is asymptomatic and C-reactive protein level is within reference range, particularly for doses less than 25 mg/day
• Rilonacept ^r16c170
  • In early 2021, the US Food and Drug Administration approved rilonacept injection for treatment of recurrent pericarditis and to reduce the risk of recurrence in adults and children 12 years and older ^r4
  • Rilonacept Solution for injection; Children and Adolescents 12 to 17 years: 4.4 mg/kg/dose (Max: 320 mg/dose) subcutaneously (1 or 2 injections, Max: 160 mg/injection) once then, 2.2 mg/kg/dose (Max: 160 mg/dose) subcutaneously once weekly starting on day 8.
  • Rilonacept Solution for injection; Adults: 320 mg subcutaneously (two 160 mg-injections) once, then 160 mg subcutaneously once weekly starting on day 8.

Nondrug and supportive care

For athletes, restrict exercise for at least 3 months after symptom resolution and normalization of C-reactive protein levels and ECG and echocardiogram findings

For nonathletes, restrict exercise until resolution of symptoms and normalization of C-reactive protein level and ECG and echocardiogram findings ^r1

Comorbidities

• Myocarditis shares a similar cause with pericarditis and therefore often occurs concurrently (15% of patients^r17) ^r1c172
  • Pericarditis with myocardial involvement is known as myopericarditis
    • Myocardial involvement may be subclinical but can be identified by increased markers of myocardial damage (ie, elevation of troponins or creatine kinase-MB) without newly developed left ventricular impairment ^r1
    • Risk factors for myocardial involvement include younger age, male sex, fever, arrhythmia, and ST-segment elevation ^r17
    • Management of myopericarditis is similar to that of pericarditis with the following exceptions: ^r1
      • There is insufficient evidence to recommend colchicine for patients with myopericarditis
      • Physical exercise should be restricted for at least 6 months from the onset of illness
    • Myopericarditis is not associated with increased risk of complications or recurrent pericarditis ^r17
• Renal disease or end-stage renal disease ^r1c173c174
  • Uremic pericarditis describes pericarditis that occurs before renal replacement therapy or within 8 weeks of initiating renal replacement therapy
  • Dialysis pericarditis occurs after a patient is stabilized on dialysis (typically 8 or more weeks after initiation)
  • Constrictive pericarditis may complicate renal disease, although very rarely
• Systemic autoimmune diseases
  • Common autoimmune comorbidities
    • Systemic lupus erythematosus ^c175
    • Sjögren syndrome ^c176
    • Rheumatoid arthritis ^c177
    • Scleroderma ^c178
  • Less common autoimmune comorbidities
    • Systemic vasculitides ^c179
    • Behçet syndrome ^c180
    • Sarcoidosis ^c181
    • Inflammatory bowel disease ^c182

Special populations

• Pregnant and breastfeeding women
  • NSAIDs
    • Classic NSAIDs (ibuprofen or indomethacin) may be used during the first and second trimesters but should be withdrawn by gestational week 32
      • Indomethacin Oral capsule; Adults: Indomethacin 75 to 200 mg/day PO divided in 3 to 4 doses has been shown to be comparable to aspirin.
    • Aspirin (100 mg/day or less) should be used after gestational week 20 because other NSAIDs may cause impaired fetal renal function
  • Corticosteroids
    • Prednisone at the lowest effective dose may be used throughout pregnancy and breastfeeding but should be supplemented with calcium (1200-1500 mg/day^r5) and vitamin D (800-1000 international units/day^r5)
  • Colchicine is contraindicated during pregnancy and breastfeeding
• Geriatric populations ^r1
  • Indomethacin is not recommended
  • Recommended to halve colchicine dose
  • Critically evaluate renal function and drug interactions before prescribing drug therapy
• Tuberculous pericarditis ^r1
  • Consider adjunctive corticosteroids in patients without HIV infection ^r1
  • Administer intrapericardial urokinase to reduce risk of progression to constrictive pericarditis
  • For patients with constrictive tuberculous pericarditis who have not improved after 4 to 8 weeks of tuberculosis therapy, pericardiectomy is indicated
• Recurrent pericarditis
  • Initially treat as for acute pericarditis
  • Treat those who do not respond to NSAIDs or who require very high doses of corticosteroids with azathioprine, IV immunoglobulin, or anakinra ^r1
  • Intrapericardial corticosteroid instillation may be an alternative to systemic corticosteroids ^r1
  • Consider pericardiectomy for patients with recurrent pericarditis refractory to all other medical therapy ^r1
• Constrictive pericarditis ^r1
  • Late sequelae of pericarditis
  • Pericardiectomy is the mainstay of treatment
  • Antiinflammatory drugs should be used for those with newly diagnosed constrictive pericarditis who are hemodynamically stable ^r1
    • May be used in patients with elevated C-reactive protein and imaging evidence of pericardial inflammation or in patients for whom surgery is contraindicated
      • May resolve constriction in 10% to 20% of patients and prevent need for surgery
    • Do not use in patients with evidence of chronic constrictive pericarditis (eg, those with cachexia, atrial fibrillation, hepatic dysfunction, or pericardial calcification)