Pericarditis in Systemic Rheumatologic Diseases
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PERICARDIAL DISEASE (L KLEIN AND CL JELLIS, SECTION EDITORS)
Pericarditis in Systemic Rheumatologic Diseases Apostolos Kontzias 1 & Amir Barkhodari 1 & QingPing Yao 1 Accepted: 3 September 2020 # Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Purpose of Review We review the epidemiology, pathophysiology, and management of pericarditis most commonly complicating autoimmune and autoinflammatory conditions. Recent Findings Typically, pericarditis occurs in the context of a systemic flare of the underlying disease but infrequently, it is the presenting manifestation requiring a high index of suspicion to unravel the indolent cause. Pericardial involvement in rheumatic diseases encompasses a clinical spectrum to include acute, recurrent and incessant pericarditis, constrictive pericarditis, asymptomatic pericardial effusion, and pericardial tamponade. Direct evidence on the pathophysiology of pericarditis in the context of rheumatic diseases is scant. It is theorized that immune perturbations within pericardial tissue result from the underlying central immunopathology of the respective autoimmune or autoinflammatory disease. Summary Pericarditis management depends on acuity, the underlying cause and epidemiological features such as patient’s immune status and geographic prevalence of infections such as tuberculosis. Immunosuppressive medications including biologics such as interleukin 1 blockers emerge as possible steroid sparing agents for pericarditis treatment. Keywords Pericarditis . Autoimmune . Autoinflammatory . Rheumatic . Pathophysiology . Treatment
Introduction Heart involvement in rheumatic diseases is common but often atypical in presentation requiring a high index of suspicion. Cardiac structures affected include the pericardium (pericarditis and pericardial effusion), the myocardium (myocarditis, cardiomyopathy, rhythm and conduction disturbances, heart failure), coronary arteries (acute coronary syndromes, ischemic heart disease), the endocardium (valvular disease, thrombi), and major vessels (aneurysm formation, arterial, and venous thrombosis) [1]. The most common types of pericardial involvement in rheumatology are acute and recurrent This article is part of the Topical Collection on Pericardial Disease * Apostolos Kontzias [email protected] Amir Barkhodari [email protected] QingPing Yao [email protected] 1
Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Stony Brook University School of Medicine, Stony Brook, NY, USA
pericarditis as well as asymptomatic pericardial effusions. A rheumatic cause is identified in 2–7% of cases of acute pericarditis and in up to 10% for recurrent pericarditis [2–4]. A systemic autoimmune disease was shown to be responsible for acute pericarditis in 7.3% in series of 453 patients [5]. In other cases, the pericardial involvement may be indolent with a clinically silent pericardial effusion [6]. Typical pericarditis symptoms include sharp midsternal chest pain up
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