Chest pain returns as prednisone is tapered. The patient has already had several evaluations, and the chart says “idiopathic recurrent pericarditis.” At this point, another question is useful: is the pericardium the main site of inflammation, or one part of a broader syndrome?
This is a teaching scenario, not an individual patient case. Many patients with recurrent pericarditis do not have a defined systemic rheumatic disease. The value of a rheumatologic evaluation is to identify meaningful patterns and guide targeted investigation.
First establish what is recurring
Recurrent chest pain alone does not establish recurrent pericardial inflammation. Reassess symptoms, examination, inflammatory markers, electrocardiography, and cardiac imaging with cardiology. Echocardiography evaluates effusion and hemodynamic consequences; cardiac magnetic resonance can help clarify inflammation in selected cases. Also consider myocardial involvement and alternative causes of pain.
Hemodynamic compromise, suspected tamponade, or other unstable findings require urgent cardiac management. Etiologic investigation should not delay stabilization.
Let the associated features organize the differential
- Connective tissue disease clues: inflammatory arthritis, photosensitive rash, oral ulcers, Raynaud phenomenon, cytopenias, or kidney findings may support investigation for lupus or another systemic autoimmune condition.
- Autoinflammatory clues: stereotyped febrile attacks, serositis at other sites, inflammatory rash, childhood symptoms, or a suggestive family history may justify evaluation for an autoinflammatory syndrome.
- Still’s disease clues: spiking fever, sore throat, an evanescent rash, arthritis, and substantial systemic inflammation should prompt consideration alongside infectious and malignant mimics.
Infection, malignancy, drug exposures, and other causes remain part of the assessment. The absence of autoantibodies is not itself a diagnosis of autoinflammation; a positive ANA is not itself a diagnosis of lupus.
A genetic result is evidence, not a verdict
Testing should answer a clinical question. A pathogenic variant with an appropriate phenotype has a different meaning from a variant of uncertain significance found on a broad panel. FMF usually follows an autosomal recessive pattern, but the interpretation of a single MEFV variant can be complex. Some carriers have inflammatory manifestations; many remain well.
Peet and colleagues studied recurrent pericarditis and monogenic autoinflammatory disease at a national referral center. Their findings support an association between recurrent pericarditis and certain MEFV variants. They do not establish that every carrier with pericarditis has FMF, or justify applying referral-center genetic frequencies to all patients with chest pain.
Treat the inflammatory problem and the underlying disease
The 2025 ESC guideline provides the current cardiac management framework. Anti-inflammatory therapy and colchicine remain central in appropriate patients; IL-1 inhibition has a role in selected recurrent inflammatory pericarditis. Choice and tapering depend on the phenotype, treatment response, contraindications, and accompanying disease. A response to IL-1 blockade does not distinguish all possible diagnoses.
If lupus, FMF, Still’s disease, or another systemic condition is established, its organ risks and long-term treatment goals must also be addressed. Repeated short courses of treatment without a shared monitoring and tapering plan can leave patients cycling between specialists.
Make the referral actionable
Send a timeline of objectively documented episodes, imaging reports, inflammatory markers during and between episodes, medication doses and taper-related relapses, and the systemic findings that prompted concern. Agree on who manages flares, refills, toxicity monitoring, and escalation. The most useful label is one that improves the plan.
Keep reading
Sources and further reading
- 2025 ESC guideline for myocarditis and pericarditis
- Peet et al. Pericarditis and autoinflammation: clinical and genetic analysis (2022)
Adapted from Jonathan S. Hausmann’s presentation, “Pericarditis in systemic disease,” with references and terminology updated for this article. Individual patient cases and slide images have not been reproduced.
This article provides general education, not a diagnosis or an individual treatment plan. Discuss personal medical decisions with your care team.