Table 1
Summary of key features of autoimmune pericarditis across the most common autoimmune conditions. NSAIDs: nonsteroidal anti-inflammatory drugs; RA: rheumatoid arthritis; DMARD: disease-modifying antirheumatic drug; IVIG: intravenous immunoglobulin; FMF: familial Mediterranean fever; TRAPS: tumor necrosis factor receptor-associated periodic syndrome
| AUTOIMMUNE CONDITION | ESTIMATED PREVALENCE OF PERICARDIAL INVOLVEMENT | TYPICAL PERICARDIAL MANIFESTATION | PATHOPHYSIOLOGIC DRIVER OF PERICARDIAL DISEASE | STANDARD MANAGEMENT | EMERGING FUTURE THERAPIES |
|---|---|---|---|---|---|
| Systemic Lupus Erythematosus | 50% asymptomatic pericardial involvement; 20-25% pericarditis | Asymptomatic involvement more prevalent than symptomatic disease. Acute or recurrent pericarditis; often painless effusions; rare tamponade or constriction | Immune-complex deposition, complement activation and interleukin-dependent cytokine amplification pathways | 1st line: NSAIDs + colchicine 2nd line: steroids 3rd line: azathioprine, mycophenolate mofetil, belimumab, and anifrolumab 4th line: IL-1 inhibitors such as rilonacept and anakinra, rituximab, cyclophosphamide, intravenous immunoglobulins | IL-1 inhibition (anakinra, rilonacept) |
| Rheumatoid Arthritis | 30-50% pericardial involvement; < 10% symptomatic pericarditis | Asymptomatic effusions more prevalent than acute disease. Acute or recurrent pericarditis; rare tamponade or constriction | Interleukin-dependent cytokine amplification pathways ± immune-complex deposition | 1st line: NSAIDs + colchicine 2nd line: steroids RA-directed DMARDs | IL-1 inhibition (anakinra, rilonacept); possible IL-6 inhibition |
| Systemic Sclerosis | Detected in autopsy series (70%); 8-fold increased risk of pericardial involvement with symptomatic effusions present in 5-16% | Small chronic effusions more common than symptomatic or acute disease | Microvascular injury, ischemia, and progressive fibrosis | 1st line: NSAIDs + colchicine 2nd line: steroids, but reduced dose for concern for scleroderma renal crises 3rd line: mycopheonlate, cyclophosphamide, rituximab, tocilizumab | Anti-fibrotic and vascular-targeted therapies |
| Mixed Connective Tissue Disease | Variable; limited epidemiologic data | Variable | Overlapping autoimmune features (eg, anti-ribonucleoprotein antibodies, interferon) | 1st line: NSAIDs + colchicine 2nd line: steroids 3rd line: mycophenolate, azathioprine, methotrexate 4th line: rituximab, cyclophosphamide, IVIG | IL-1 inhibition (anakinra, rilonacept) |
| Vasculitis | Uncommon overall; 1-3% pericardial involvement; myocarditis and valvular disease are more common | Inflammation of pericardial walls causing acute presentation | Less defined; can be cell or immune complex mediated | 1st line: NSAIDs + colchicine 2nd line: steroids Treat underlying vasculitis | IL-1 inhibition (anakinra, rilonacept) |
| Autoinflammatory Conditions (eg, FMF, TRAPS) | Rare but recognized | Recurrent inflammatory pericarditis | Innate immune activation | 1st line: colchicine for FMF, colchicine/NSAIDs/steroids for TRAPS | IL-1 inhibition (anakinra, rilonacept) |