Pericardial involvement in systemic autoimmune diseases
- Massimo Imazio, MD, FESC
Massimo Imazio, MD, FESC
- Professor of Physiology
- University Division of Cardiology, Department of Medical Sciences, AOU Città della Salute e della Scienza di Torino.
- Department of Public Health and Pediatrics, University of Torino.
Systemic autoimmune diseases, also known as systemic inflammatory diseases, are inflammatory syndromes involving at least two organ systems. Classical systemic inflammatory diseases include connective tissue diseases, vasculitis, and granulomatous diseases (table 1) . Cardiac involvement is not uncommon in systemic inflammatory diseases, although major cardiac problems are not usually the presenting manifestation. The reported frequency of cardiac involvement is variable depending on the applied diagnostic methods and patient selection . Technological improvements in imaging modalities and the increasing availability of diagnostic imaging have revealed a higher frequency of cardiac abnormalities in patients with systemic inflammatory diseases than in older autopsy studies.
Systemic inflammatory diseases may affect the pericardium (pericarditis and pericardial effusion), myocardium (myocarditis, cardiomyopathy, rhythm and conduction disturbances, heart failure), coronary arteries (acute coronary syndromes, ischemic heart disease), endocardium (valvular disease, thrombi), and major vessels (aneurysm formation, arterial and venous thrombosis) [1,2]. On rare occasions, pericardial effusion and/or pericarditis may be the first clues of a systemic inflammatory disease or a manifestation of a known systemic disease. In these cases, pericardial involvement generally reflects the activity of the systemic disease.
This topic will focus on the pericardial complications of systemic autoimmune diseases. A detailed discussion of non-pericardial cardiac manifestations of systemic autoimmune diseases is presented separately. (See "Non-coronary cardiac manifestations of systemic lupus erythematosus in adults" and "Overview of the systemic and nonarticular manifestations of rheumatoid arthritis", section on 'Cardiac disease' and "Cardiac sarcoidosis".)
CLINICAL PRESENTATION, DIAGNOSIS, AND TREATMENT
The most common types of pericardial involvement with systemic inflammatory diseases are acute (or recurrent) pericarditis and asymptomatic pericardial effusions. Pericardial involvement in systemic inflammatory disease is thought to be immune-mediated, although concomitant infection may play a role in some cases. Systemic inflammatory diseases may be either "etiologic" or "permissive" of increased susceptibility to an unrelated primary cause (eg, viral) .
While some patients may manifest with the typical signs and symptoms of pericarditis (fever, pleuritic chest pain) or a hemodynamically significant effusion (dyspnea, fatigue, hypotension), many are incidentally found to have pericardial involvement during other testing (electrocardiography, echocardiography, computed tomography of the chest, or magnetic resonance imaging of the heart). Symptoms of pericardial involvement in systemic inflammatory disease may be insidious or may present suddenly with rapid progression. Cardiac tamponade and constrictive pericarditis are possible, but rare, complications. (See "Clinical presentation and diagnostic evaluation of acute pericarditis", section on 'Clinical features' and "Diagnosis and treatment of pericardial effusion", section on 'Diagnostic approach'.)
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- CLINICAL PRESENTATION, DIAGNOSIS, AND TREATMENT
- SPECIFIC SYSTEMIC INFLAMMATORY DISEASES WITH PERICARDIAL INVOLVEMENT
- Connective tissue diseases
- - Systemic lupus erythematosus
- - Rheumatoid arthritis
- - Adult Still's disease
- - Systemic sclerosis
- - Dermatomyositis and polymyositis
- - Mixed connective tissue disease
- - Sjögren's syndrome
- Other disease entities
- - Sarcoidosis
- - Pericarditis in acute rheumatic fever
- - Vasculitis
- Behçet's syndrome
- AUTOINFLAMMATORY DISEASES
- Familial Mediterranean Fever
- Tumor necrosis factor receptor-1 associated periodic syndrome (TRAPS)
- SUMMARY AND RECOMMENDATIONS