What are the symptoms and treatment options for pericarditis, particularly in patients with a history of autoimmune disorders such as rheumatoid arthritis or lupus?

Symptoms of Pericarditis

Clinical Presentation

Pericarditis presents with characteristic sharp, pleuritic chest pain that worsens when supine and improves when leaning forward, occurring in approximately 90% of patients. 1

The diagnosis requires at least 2 of the following 4 clinical criteria:

• Pericarditic chest pain - sharp, pleuritic quality that radiates to the left shoulder and is relieved by sitting up and leaning forward 1, 2
• Pericardial friction rub - present in less than 30% of cases, making it an insensitive but specific finding 1, 2
• New widespread ST-elevation or PR depression on ECG - seen in 25-60% of cases, though the pericardium itself is electrically inert and these changes reflect epicardial inflammation 1, 2
• New or worsening pericardial effusion - detected in approximately 60% of cases, most often small 1, 2

Supporting Findings

Additional features that support the diagnosis include:

• Elevated inflammatory markers - C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and white blood cell count are commonly elevated and useful for monitoring disease activity 1
• Fever - temperature >38°C (>100.4°F) indicates higher risk and warrants hospital admission 3
• Advanced imaging findings - CT or cardiac MRI can provide additional diagnostic confirmation 1

Special Considerations in Autoimmune Disease

In patients with rheumatoid arthritis or lupus, pericarditis typically occurs during a systemic flare of the underlying disease, though it rarely presents as the first manifestation. 1

Key features in autoimmune-related pericarditis:

• Pericardial involvement is common in systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, and scleroderma, affecting approximately 5-15% of patients with acute or recurrent pericarditis 1
• Concomitant myocardial involvement should be ruled out, as myopericarditis can complicate the presentation 1
• Asymptomatic pericardial effusion may occur without typical chest pain symptoms 1

Atypical Presentations in Special Populations

Patients with end-stage renal disease present atypically, with only 30% experiencing pleuritic chest pain and most lacking ECG abnormalities due to absence of myocardial inflammation. 1

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Treatment Approach

First-Line Therapy

High-dose NSAIDs combined with colchicine for 3 months is the recommended first-line treatment for acute pericarditis. 4

NSAID Dosing

• Aspirin 750-1000 mg every 8 hours OR Ibuprofen 600 mg every 8 hours for 1-2 weeks with gastroprotection 4
• Continue at full doses until complete symptom resolution 1, 4
• Taper gradually after CRP normalization: decrease aspirin by 250-500 mg every 1-2 weeks 4

Colchicine Dosing

• 0.5 mg twice daily for patients ≥70 kg 4
• 0.5 mg once daily for patients <70 kg 4
• Duration: 3 months for first episode 4
• Duration: 6 months for recurrent pericarditis 5

Activity Restriction

• Exercise restriction until symptoms resolve and CRP, ECG, and echocardiogram normalize 4
• Athletes require at least 3 months of restriction 4

Monitoring Treatment Response

CRP levels should guide treatment duration and tapering decisions. 4, 5

• Taper medications only when symptoms are absent and CRP is normal 4
• Stop one drug class at a time during tapering 5

Second-Line Therapy

Low-dose corticosteroids (prednisone 0.2-0.5 mg/kg/day) should be considered only when NSAIDs/colchicine are contraindicated, fail to control symptoms, or after infectious causes have been excluded. 4

Critical Warnings About Corticosteroids

• NOT recommended as first-line therapy due to increased risk of chronicity and recurrence 4
• Use the lowest effective dose to minimize side effects 1
• Specific indications include autoimmune disease, pregnancy, or contraindications to NSAIDs/colchicine 5

Corticosteroid Tapering Protocol 1

Starting Dose	Tapering Schedule
>50 mg	Decrease 10 mg/day every 1-2 weeks
50-25 mg	Decrease 5-10 mg/day every 1-2 weeks
25-15 mg	Decrease 2.5 mg/day every 2-4 weeks
<15 mg	Decrease 1.25-2.5 mg/day every 2-6 weeks

• Bone protection required: Calcium 1200-1500 mg/day plus vitamin D 800-1000 IU/day for all patients on corticosteroids 1
• Bisphosphonates recommended for men ≥50 years and postmenopausal women on ≥5.0-7.5 mg/day prednisone 1

Treatment in Autoimmune Disease Patients

Treatment should be targeted at controlling the underlying systemic disease in patients with rheumatoid arthritis or lupus. 1

• Consultation with rheumatology or immunology is warranted when autoimmune disease is suspected 1
• The degree of pericardial involvement generally reflects the activity of the underlying autoimmune condition 1

Third-Line Therapy for Refractory Cases

For corticosteroid-dependent recurrent pericarditis not responsive to colchicine, consider IL-1 blockers (anakinra), IVIG, or azathioprine. 1, 5

• These agents require multidisciplinary consultation with immunologists and/or rheumatologists 1
• Patient education about immunosuppressive risks is mandatory 1
• Pericardiectomy is a last resort after exhaustive medical therapy failure, performed only at centers with specific expertise 1

Common Pitfalls

Inadequate treatment of the first episode is the most common cause of recurrence. 4

• Recurrence rates: 15-30% without colchicine, increasing to 50% after first recurrence 4
• Premature tapering before CRP normalization increases recurrence risk 4
• Corticosteroids provide rapid symptom control but may paradoxically increase chronicity 4

Prognostic Information

Constrictive pericarditis risk is very low (<1%) in idiopathic/viral pericarditis but higher (2-5%) in autoimmune/neoplastic causes and highest (20-30%) in bacterial pericarditis. 4

• Cardiac tamponade rarely occurs in idiopathic pericarditis (<3%) but is more common with malignancy or purulent pericarditis 4, 2
• With appropriate treatment, 70-85% of patients have a benign course 2