11 A Toolkit for Health Care Professionals Recurrent Pericarditis Differential Diagnosis The differential diagnosis of those presenting with pericarditis is dependent upon signs and symptoms, prior medical history and acuity. In acute pericarditis, chest pain occurs >95% of the time.29 Myocardial ischemia (including ST-segment elevation myocardial infarction), aortic dissection, myocarditis (without a pericardial component) and pulmonary embolism should be ruled out or deemed less likely. Although the age of presentation may influence diagnostic decision-making, up to 30% of myocardial infarctions occur in those <55 years of age,30 and thus either noninvasive (e.g., computed tomography [CT]) or invasive (angiography) imaging may be required, especially if the ECG changes are not diagnostic of pericarditis. Given the inflammatory state of pericarditis (with associated leukocytosis and fever), the differential diagnosis of pericarditis often includes infections such as pneumonia. Other common non-cardiac causes of acute chest pain consist of gastrointestinal disorders, such as acid reflux, musculoskeletal complaints and fibromyalgia. These conditions can be misdiagnosed as pericarditis, particularly in those with ECGs consistent with early repolarization or left ventricular hypertrophy. In cases of suspected recurrent pericarditis, where the chest pain syndrome may be less severe, normal inflammatory biomarkers and nondiagnostic ancillary imaging should prompt a search for non-pericardial chest pain etiologies. Patients who present with a pericardial effusion without a high clinical suspicion of pericarditis may at times present a diagnostic dilemma. In a retrospective analysis of 269 patients with a pericardial effusion requiring pericardiocentesis: 26% were idiopathic 25% were malignant (including 9% as a first diagnosis of a malignancy) 20% were iatrogenic The rest of the various etiologies included heart failure, uremia, systemic disease and infection.31 The underlying causes of pericarditis can be coarsely divided into idiopathic (often assumed to be viral), infectious and noninfectious etiologies. An antecedent upper respiratory or gastrointestinal tract infection was identified in up to 40% of patients presenting with acute pericarditis without a known etiology.32 In a series of 1,162 patients with pericarditis: 55% were deemed idiopathic 9% were neoplastic 2.6% were autoimmune.33 Viruses implicated in pericarditis include coxsackievirus, adenovirus, influenza, HIV and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). In developed countries, bacterial and TB-driven pericarditis is rare; however, in developing countries with a high TB prevalence, this is a common entity. Other etiologies include radiationinduced and post-cardiac injury syndrome. Often, pericarditis and, specifically, recurrent pericarditis cases will prompt a workup for an autoimmune etiology. More common autoimmune diseases associated with pericarditis include systemic lupus erythematosus, rheumatoid arthritis and systemic sclerosis, although rarely is pericardial involvement implicated as the first clinical presentation of an autoimmune disease. Autoinflammatory disorders are an emerging cause of acute and recurrent pericarditis. Genetic data links monogenic mutations, such as Mediterranean Fever (MEFV) (implicated in Familial Mediterranean Fever) and others, including inflammasome-related mutations, to recurrent pericarditis.34 Concordant with this, a genomewide association study of almost 5,000 individuals with acute and recurrent pericarditis found an association between pericarditis and sequence variants at the interleukin 1 gene locus.35 These studies suggest that those with previously presumed idiopathic and/or viral-induced pericarditis may actually hold a genetic predisposition.
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