Pericarditis

History

• Sudden onset of retrosternal pleuritic chest pain is most common presenting symptom ^r8c1
  • 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: ^r8
  • Fever (more common in children than adults) ^r1c8
    • Fevers due to bacterial pericarditis occur at regular intervals and are associated with frank rigors ^r8
  • Malaise and myalgia may be present ^r9c9c10
  • If pericardial effusion develops, dyspnea, dry cough, dysphagia, hiccups, and dysphonia may result ^c11c12c13c14
  • 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 ^r9c15
• 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 ^r8c18
• Pericardial friction rub may be audible on auscultation over the left sternal border (35% of patients) ^r8c19
  • 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 ^r2
  • 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 ^r3
• 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 ^r8c22
  • 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: ^r8
  • 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% to 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
        • Postmyocardial 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, thiazide diuretics, 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 ^c122
  • Chest radiography ^r2c123
  • Laboratory studies ^r3
    • CBC with differential ^c124
    • C-reactive protein and/or erythrocyte sedimentation rate ^c125c126
    • Cardiac troponin level ^c127
    • Renal function and liver tests ^c128c129
  • Transthoracic echocardiography ^c130
• 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) ^c131
  • Cardiac CT scan or MRI in patients with suspected constrictive pericarditis, nondiagnostic echocardiography, or complicated course ^c132c133
  • Tuberculosis testing (eg, interferon-γ release assay; culture of sputum, gastric aspirate, and/or urine for Mycobacterium tuberculosis) and chest CT scan in patients suspected of having tuberculous pericarditis (eg, those who reside in tuberculosis-endemic areas, immunocompromised patients) ^c134
  • HIV serology ^c135
  • 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 ^c136c137c138
  • Consider specific neoplastic markers and CT scan of chest and abdomen for patients with suspected neoplastic pericarditis ^c139c140
  • Pericardiocentesis for patients with suspected bacterial, neoplastic, or refractory pericarditis; perform therapeutically in patients with cardiac tamponade ^c141
  • 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 ^c142
  • Cardiac catheterization can be used to diagnose constrictive pericarditis when other diagnostic methods are inconclusive ^r1c143
• 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 ^c144
  • Characterized by leukocytosis ^r1
• Inflammatory markers
  • Characteristically associated with elevated erythrocyte sedimentation rate ^c145
  • 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 ^r13c146
• Cardiac troponin levels
  • Troponin I and T levels are elevated in 35% to 50% of acute pericarditis cases as a result of epicardial inflammation ^r14c147c148
  • Correlates with ST-segment elevation on ECG ^r14
  • 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 ^r14

Imaging

• Chest radiography ^r1c149
  • 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 suggest lymphadenopathy from tuberculous or fungal pericarditis
  • May show pericardial calcifications suggestive of constrictive pericarditis ^r1
• Transthoracic echocardiography ^r1c150
  • 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 ^r3
  • Able to identify disease complicated by myocardial involvement ^r2
  • 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 to 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 scan ^r1c151
  • 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 scan 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 ^c152
  • 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 scan; 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 ^c153
  • 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 ^r8
    • 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 ^r8
      • Typically seen within hours to days of chest pain onset ^r8
    • Stage 2: ST and PR segments normalize ^r8
      • Typically occurs within the first week of illness ^r3
    • Stage 3: symmetrical, widespread T-wave inversions ^r8
    • Stage 4: findings may appear normal or T-wave inversions persist indefinitely ^r8
  • 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 ^c156c157d1
  • 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 ^c158d2
  • Presentation includes fever and productive cough; chest pain and dyspnea may occur
  • Differentiated based on clinical findings and chest radiograph
• Pulmonary embolism ^c159d3
  • 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 ^c160
  • 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) ^c161
  • 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 ^c162
  • 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 ^c163c164c165
  • Aspirin
    • For acute pericarditis
      • 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.
    • For recurrent pericarditis
      • 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
    • For acute pericarditis
      • Ibuprofen Oral tablet; Adults: 600 to 800 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.
    • For recurrent pericarditis
      • Ibuprofen Oral tablet; Adults: 600 to 800 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
    • For acute pericarditis
      • 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.
    • For recurrent pericarditis
      • 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 ^c166
  • NOTE: The 0.5-mg tablet of colchicine is not always readily available. The 0.6-mg tablet or dose can be used in place of the guideline-recommended 0.5-mg dosing
  • For acute pericarditis
    • Colchicine Oral tablet; Adults 18 to 70 years weighing less than 70 kg: 0.5 or 0.6 mg PO once daily for 3 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.5 or 0.6 mg PO every other day in the last weeks.
    • Colchicine Oral tablet; Adults 18 to 70 years weighing 70 kg or more: 0.5 or 0.6 mg PO twice daily for 3 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.5 or 0.6 mg PO once daily in the last weeks.
    • Colchicine Oral tablet; Adults older than 70 years weighing less than 70 kg: 0.25 or 0.3 mg PO once daily for 3 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.25 or 0.3 mg PO every other day in the last weeks.
    • Colchicine Oral tablet; Adults older than 70 years weighing 70 kg or more: 0.25 or 0.3 mg PO twice daily for 3 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.25 or 0.3 mg PO once daily in the last weeks.
  • For recurrent pericarditis
    • Colchicine Oral tablet; Adults 18 to 70 years weighing less than 70 kg: 0.5 or 0.6 mg PO once daily for at least 6 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.5 or 0.6 mg PO every other day in the last weeks.
    • Colchicine Oral tablet; Adults 18 to 70 years weighing 70 kg or more: 0.5 or 0.6 mg PO twice daily for at least 6 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.5 or 0.6 mg PO once daily in the last weeks.
    • Colchicine Oral tablet; Adults older than 70 years weighing less than 70 kg: 0.25 or 0.3 mg PO once daily for at least 6 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.25 or 0.3 mg PO every other day in the last weeks.
    • Colchicine Oral tablet; Adults older than 70 years weighing 70 kg or more: 0.25 or 0.3 mg PO twice daily for at least 6 months in combination with aspirin/NSAID. Tapering colchicine to discontinue is not mandatory; may decrease dose to 0.25 or 0.3 mg PO once daily in the last weeks.
• Corticosteroids
  • Prednisone ^c167
    • For acute pericarditis
      • Prednisone Oral tablet; Adults: 0.2 to 0.5 mg/kg/dose PO once daily for 2 to 4 weeks in combination with colchicine until asymptomatic and CRP concentration is normal, then taper dose by 5 to 10 mg/day every 1 to 2 weeks for doses more than 25 mg/day, by 2.5 mg/day every 2 to 4 weeks for doses of 15 to 25 mg/day, and then by 1.25 to 2.5 mg/day every 2 to 6 weeks for doses less than 15 mg/day.
    • For recurrent pericarditis
      • Prednisone Oral tablet; Adults: 0.2 to 0.5 mg/kg/dose PO once daily for 2 to 4 weeks in combination with aspirin/NSAID and colchicine until asymptomatic and CRP concentration is normal, then taper dose by 5 to 10 mg/day every 1 to 2 weeks for doses more than 25 mg/day, by 2.5 mg/day every 2 to 4 weeks for doses of 15 to 25 mg/day, and then by 1.25 to 2.5 mg/day every 2 to 6 weeks for doses less than 15 mg/day.
• Interleukin-1 receptor antagonist
  • Rilonacept ^c168
    • Rilonacept Solution for injection; Adults: 320 mg subcutaneously 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^r22) ^r1c170
  • 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 postmyocardial infarction pericarditis) without newly developed left ventricular impairment ^r1
    • Risk factors for myocardial involvement include younger age, male sex, fever, arrhythmia, and ST-segment elevation ^r22
    • 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 ^r22
• Renal disease or end-stage renal disease ^r1c171c172
  • 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 ^c173
    • Sjögren syndrome ^c174
    • Rheumatoid arthritis ^c175
    • Scleroderma ^c176
  • Less common autoimmune comorbidities
    • Systemic vasculitides ^c177
    • Behçet syndrome ^c178
    • Sarcoidosis ^c179
    • Inflammatory bowel disease ^c180

Special populations

• Pregnant and breastfeeding women
  • NSAIDs
    • Aspirin and other NSAIDs (ibuprofen preferred) may be used as needed during the first and second trimesters; only low dose aspirin permitted during third trimester ^r23
  • Corticosteroids ^r23
    • Prednisone at the lowest effective dose for the least amount of time may be used throughout pregnancy and breastfeeding
  • Colchicine may be used at any point during pregnancy to reduce the risk of recurrent pericarditis ^r23
  • For incessant, recurrent, or refractory pericarditis or when other therapies are contraindicated, therapy with IL-1 inhibitor can be considered; safety data are limited in this population ^r23
• 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
  • 2015 ESC guidelines recommend treating those who do not respond to NSAIDs or who require very high doses of corticosteroids with azathioprine, IV immunoglobulin, or anakinra ^r1
    • Data published since those guidelines show much stronger evidence in favor of both anakinra and rilonacept ^r6
  • 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)