First line treatment for a first episode (all drugs together with gastric protection):
- Aspirin 750 to 1000 mg orally every 8 hours (maximum 4 g daily) for 1 to 2 weeks, then reduce by 250 to 500 mg every 1 to 2 weeks until stopped (total course 3 to 4 weeks).
- Or ibuprofen 600 mg orally every 8 hours (maximum 2400 mg daily) for 1 to 2 weeks, reducing by 200 to 400 mg every 1 to 2 weeks.
- Continue until symptoms resolve and C-reactive protein is normal. Avoid indometacin in older adults and in coronary disease. Monitor creatinine, blood pressure and gastrointestinal bleeding.
- Avoid in heart failure with reduced ejection fraction, eGFR under 30 mL/min/1.73 m2, active peptic ulcer, and with anticoagulants unless benefit clearly outweighs risk.
- Omeprazole 20 mg orally once daily while on NSAIDs.
- Colchicine 0.5 mg orally once daily (weight under 70 kg) or 0.5 mg twice daily (weight 70 kg or over), without a loading dose, for 3 months for a first episode and at least 6 months for recurrence.
- Check creatinine, liver tests and creatine kinase at baseline and at 1 month.
- Reduce colchicine to 0.5 mg daily (or 0.5 mg on alternate days) for eGFR 30 to 50 mL/min/1.73 m2 and avoid at eGFR under 30 or in severe hepatic impairment.
- Avoid strong CYP3A4 or P-glycoprotein inhibitors (clarithromycin, ketoconazole, itraconazole, ciclosporin; with verapamil or diltiazem reduce the dose). Stop for diarrhoea, myopathy or cytopenias.
- Restrict physical activity until symptoms resolve and C-reactive protein is normal (non-athletes), and for at least 3 months in athletes.
Treatment tied to the underlying disease.
- Systemic lupus erythematosus:
- ibuprofen 600 mg orally every 8 hours (maximum 2400 mg daily) with colchicine 0.5 mg orally once or twice daily by weight
- hydroxychloroquine 200 to 400 mg orally daily (maximum 5 mg/kg of actual body weight daily; baseline and yearly retinal screening after 5 years; reduce in eGFR under 30 mL/min/1.73 m2)
- prednisolone 0.25 to 0.5 mg/kg orally daily for moderate or severe pericarditis, with slow taper
- for steroid sparing therapy, azathioprine 1 to 2.5 mg/kg daily (check thiopurine methyltransferase activity; full blood count and liver tests every 2 weeks for 2 months, then every 3 months) or mycophenolate mofetil 500 mg twice daily increased to 1 to 1.5 g twice daily
- Rheumatoid arthritis, systemic sclerosis, Sjogren syndrome and vasculitis: treat the underlying disease under rheumatology direction; the same pericarditis drugs apply.
- Familial Mediterranean fever: colchicine 1 to 2 mg orally daily (higher doses split), life long; anakinra when colchicine resistant.
Corticosteroids. Not first line for idiopathic pericarditis because they raise recurrence. They are used when NSAIDs and colchicine are contraindicated or have failed, and for connective tissue disease flares.
Prednisolone 0.2 to 0.5 mg/kg orally daily (not 1 mg/kg) for 2 to 4 weeks, then a slow taper:
- above 50 mg: decrease by 10 mg every 1 to 2 weeks
- 25 to 50 mg: decrease by 5 to 10 mg every 1 to 2 weeks
- 15 to 25 mg: decrease by 2.5 mg every 2 to 4 weeks
- under 15 mg: decrease by 1.25 to 2.5 mg every 2 to 6 weeks
Add bone protection and monitor glucose, blood pressure and infection. Never taper while symptoms or C-reactive protein are active. Exclude tuberculosis first in endemic regions.
Recurrent or refractory pericarditis (relapse while on or after therapy).
- Restart the effective anti-inflammatory drug at a full dose, with colchicine, and taper more slowly.
- Anakinra 100 mg subcutaneously once daily, long term (usually 6 months to 1 year or more, then taper).
- On anakinra, monitor full blood count (neutropenia), liver tests, injection site reactions and infection. Reduce to 100 mg on alternate days in eGFR under 30 mL/min/1.73 m2. Screen for tuberculosis before starting, and avoid live vaccines.
- Rilonacept 320 mg subcutaneously as a loading dose, then 160 mg once weekly (licensed for recurrent pericarditis in some regions); monitor for infection, injection site reactions and lipid changes.
- Others for steroid dependent disease: azathioprine 1 to 3 mg/kg orally daily, intravenous immunoglobulin 400 to 500 mg/kg daily for 5 days, methotrexate 10 to 15 mg orally once weekly with folic acid 5 mg once weekly (avoid in eGFR under 30 mL/min/1.73 m2).
- Pericardiectomy only for refractory disease or constriction, after exhausting medical options.
Effusion and tamponade. Drain for tamponade or when the cause remains unknown and the effusion is large (over 20 mm in diastole); send fluid for cell count, biochemistry, culture, tuberculosis testing and cytology.
Constrictive pericarditis may develop and is treated with diuretics, anti-inflammatory therapy in the transient form, and pericardiectomy when permanent.
Monitoring.
- Symptoms, C-reactive protein and ECG at 1 to 2 weeks, 4 weeks and at the end of treatment.
- Echocardiography for effusion at 1 to 2 weeks and 3 months.
- Blood count, creatinine and liver tests on colchicine and immunosuppression.
- Clinical review every 3 months for 1 year in recurrent disease.
Referral and escalation. Refer to rheumatology for connective tissue disease or autoinflammatory disease. Refer urgently for:
- tamponade
- haemodynamic compromise
- myopericarditis with arrhythmia or left ventricular dysfunction
- failure to respond to NSAIDs after 1 week
- constriction
Refer recurrent disease to a pericardial disease or specialist cardiology service.
Special groups.
- Pregnancy:
- low dose aspirin or ibuprofen until week 20 only (avoid NSAIDs after week 20 because of ductal closure and oligohydramnios)
- prednisolone 5 to 20 mg daily is acceptable
- hydroxychloroquine is continued in lupus
- colchicine, anakinra and rilonacept are avoided where alternatives exist, though colchicine is used in familial Mediterranean fever
- azathioprine is acceptable
- methotrexate and mycophenolate are contraindicated
- Breastfeeding: aspirin avoided, ibuprofen acceptable, prednisolone acceptable, colchicine considered compatible at usual doses with limited data.
- Children: ibuprofen 10 mg/kg orally every 6 to 8 hours (maximum 2400 mg daily); colchicine and anakinra dosing by paediatric specialist.
- Kidney and liver impairment: avoid NSAIDs when eGFR is under 30 mL/min/1.73 m2; reduce colchicine to 0.5 mg daily (or on alternate days) at eGFR 30 to 50 and avoid it under 30 or in severe hepatic impairment.
- Region specific: tuberculosis and HIV are leading causes of pericardial disease in sub-Saharan Africa, so exclude tuberculosis before steroids or biologics and test for HIV in every patient.