UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Pericardial involvement in systemic autoimmune diseases

Author
Massimo Imazio, MD, FESC
Section Editor
Jae K Oh, MD
Deputy Editor
Brian C Downey, MD, FACC

INTRODUCTION

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) [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 [1]. 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) [2].

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'.)

                  

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Mon Sep 29 00:00:00 GMT+00:00 2014.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Knockaert DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J 2007; 28:1797.
  2. Spodick DH. Pericardial disease in the vasculitis-connective tissue disease group. In: The Pericardium. A comprehensive textbook, Marcel Dekker, New York 1997. p.314-33.
  3. Imazio M, Spodick DH, Brucato A, et al. Controversial issues in the management of pericardial diseases. Circulation 2010; 121:916.
  4. Scardapane A, Brucato A, Chiarelli F, Breda L. Efficacy of an interleukin-1β receptor antagonist (anakinra) in idiopathic recurrent pericarditis. Pediatr Cardiol 2013; 34:1989.
  5. Vassilopoulos D, Lazaros G, Tsioufis C, et al. Successful treatment of adult patients with idiopathic recurrent pericarditis with an interleukin-1 receptor antagonist (anakinra). Int J Cardiol 2012; 160:66.
  6. Picco P, Brisca G, Traverso F, et al. Successful treatment of idiopathic recurrent pericarditis in children with interleukin-1beta receptor antagonist (anakinra): an unrecognized autoinflammatory disease? Arthritis Rheum 2009; 60:264.
  7. Haley JH, Tajik AJ, Danielson GK, et al. Transient constrictive pericarditis: causes and natural history. J Am Coll Cardiol 2004; 43:271.
  8. Langley RL, Treadwell EL. Cardiac tamponade and pericardial disorders in connective tissue diseases: case report and literature review. J Natl Med Assoc 1994; 86:149.
  9. Doria A, Iaccarino L, Sarzi-Puttini P, et al. Cardiac involvement in systemic lupus erythematosus. Lupus 2005; 14:683.
  10. Imazio M, Brucato A, Trinchero R, et al. Colchicine for pericarditis: hype or hope? Eur Heart J 2009; 30:532.
  11. Tincani A, Rebaioli CB, Taglietti M, Shoenfeld Y. Heart involvement in systemic lupus erythematosus, anti-phospholipid syndrome and neonatal lupus. Rheumatology (Oxford) 2006; 45 Suppl 4:iv8.
  12. Voskuyl AE. The heart and cardiovascular manifestations in rheumatoid arthritis. Rheumatology (Oxford) 2006; 45 Suppl 4:iv4.
  13. Efthimiou P, Paik PK, Bielory L. Diagnosis and management of adult onset Still's disease. Ann Rheum Dis 2006; 65:564.
  14. Parvez N, Carpenter JL. Cardiac tamponade in Still disease: a review of the literature. South Med J 2009; 102:832.
  15. Byers RJ, Marshall DA, Freemont AJ. Pericardial involvement in systemic sclerosis. Ann Rheum Dis 1997; 56:393.
  16. Meune C, Vignaux O, Kahan A, Allanore Y. Heart involvement in systemic sclerosis: evolving concept and diagnostic methodologies. Arch Cardiovasc Dis 2010; 103:46.
  17. Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003; 362:971.
  18. Bazzani C, Cavazzana I, Ceribelli A, et al. Cardiological features in idiopathic inflammatory myopathies. J Cardiovasc Med (Hagerstown) 2010; 11:906.
  19. Lundberg IE. Cardiac involvement in autoimmune myositis and mixed connective tissue disease. Lupus 2005; 14:708.
  20. Gyöngyösi M, Pokorny G, Jambrik Z, et al. Cardiac manifestations in primary Sjögren's syndrome. Ann Rheum Dis 1996; 55:450.
  21. Wyplosz B, Marijon E, Dougados J, Pouchot J. Sarcoidosis: an unusual cause of acute pericarditis. Acta Cardiol 2010; 65:83.
  22. Narita H, Ohte N, Yoneyama A, et al. Takayasu's arteritis accompanied with massive pericardial effusion--a case report. Angiology 1999; 50:421.
  23. Li JJ, Fang CH, Chen MZ, Chen X. Takayasu's arteritis accompanied with pericarditis: a case report. Cardiology 2004; 102:106.
  24. Guillaume M, Vachiery F, Cogan E. Pericarditis: an unusual manifestation of giant cell arteritis. Am J Med 1991; 91:662.
  25. Gonzalez-Gay MA, Martinez-Dubois C, Agudo M, et al. Giant cell arteritis: epidemiology, diagnosis, and management. Curr Rheumatol Rep 2010; 12:436.
  26. Blot M, Guépet H, Aubriot-Lorton MH, et al. An atypical case of giant cell arteritis (Horton's disease) associated with facial swelling, confusion, and pericarditis in an elderly woman. J Am Geriatr Soc 2010; 58:2040.
  27. Cullen S, Duff DF, Denham B, Ward OC. Cardiovascular manifestations in Kawasaki disease. Ir J Med Sci 1989; 158:253.
  28. Agard C, Rendu E, Leguern V, et al. Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature. Semin Arthritis Rheum 2007; 36:386.
  29. Korantzopoulos P, Papaioannides D, Siogas K. The heart in Wegener's granulomatosis. Cardiology 2004; 102:7.
  30. Baldini C, Talarico R, Della Rossa A, Bombardieri S. Clinical manifestations and treatment of Churg-Strauss syndrome. Rheum Dis Clin North Am 2010; 36:527.
  31. Atzeni F, Sarzi-Puttini P, Doria A, et al. Behçet's disease and cardiovascular involvement. Lupus 2005; 14:723.
  32. Rigante D, Cantarini L, Imazio M, et al. Autoinflammatory diseases and cardiovascular manifestations. Ann Med 2011; 43:341.
  33. Kees S, Langevitz P, Zemer D, et al. Attacks of pericarditis as a manifestation of familial Mediterranean fever (FMF). QJM 1997; 90:643.
  34. Okutur K, Seber S, Oztekin E, et al. Recurrent pericarditis as the initial manifestation of Familial Mediterranean fever. Med Sci Monit 2008; 14:CS139.
  35. Cantarini L, Lucherini OM, Cimaz R, et al. Idiopathic recurrent pericarditis refractory to colchicine treatment can reveal tumor necrosis factor receptor-associated periodic syndrome. Int J Immunopathol Pharmacol 2009; 22:1051.
  36. Cantarini L, Lucherini OM, Baldari CT, et al. Familial clustering of recurrent pericarditis may disclose tumour necrosis factor receptor-associated periodic syndrome. Clin Exp Rheumatol 2010; 28:405.