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Autoimmune Pericarditis: Multimodality Imaging

  • Pericardial Disease (AL Klein and CL Jellis, Section Editors)
  • Published:
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Abstract

Purpose of the Review

The purpose of this review is to understand the underlying mechanism that leads to pericarditis in systemic autoimmune and autoinflammatory diseases. The underlying mechanism plays a vital role in the appropriate management of patients. In addition, we will review the current landscape of available cardiac imaging modalities with emphasis on pericardial conditions as well as proposed treatment and management tailored toward pericardial autoimmune and autoinflammatory processes.

Recent Findings

Approximately 22% of all cases of pericarditis with a known etiology are caused by systemic autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis, and vasculitis. In recent years, there have been advancements of imaging modalities including cardiac MRI, cardiac CT scan, and PET scan and their respective nuances in regard to contrast use, technique, and views which clinicians may utilize to better understand the extent of a patient’s pericardial pathology and the trajectory of his or her disease process.

Summary

In this review, we will discuss systemic autoimmune and autoinflammatory diseases that involve the pericardium. We will also review different imaging modalities that are currently used to further characterize such conditions. Having a deeper understanding of such techniques will improve patient outcomes by helping clinicians tailor treatment plans according to the unique underlying condition.

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References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Hoit BD. Anatomy and physiology of the pericardium. Cardiol Clin. 2017;35:481–90.

    Article  Google Scholar 

  2. Little WC, Freeman GL. Pericardial disease. Circulation. 2006;113:1622–32.

    Article  Google Scholar 

  3. • Imazio M, Gaita F, LeWinter M. Evaluation and treatment of pericarditis: a systematic review. JAMA. 2015;314:1498–506. This paper highlights the diagnostic steps involved in evaluating pericarditis and summarizes the treatment options available for this condition.

    Article  CAS  Google Scholar 

  4. Adler Y, Charron P, Imazio M, et al. 2015 ESC Guidelines for the diagnosis and management of pericardial diseases: the Task Force for the Diagnosis and Management of Pericardial Diseases of the European Society of Cardiology (ESC) Endorsed by: The European Association for Cardio-Thoracic Surgery (EACTS). Eur Heart J. 2015;36:2921–64.

    Article  Google Scholar 

  5. Imazio M, Brucato A, Doria A, Brambilla G, Ghirardello A, Romito A, Natale G, Palmieri G, Trinchero R, Adler Y. Antinuclear antibodies in recurrent idiopathic pericarditis: prevalence and clinical significance. Int J Cardiol. 2009;136:289–93.

    Article  Google Scholar 

  6. Zayas R, Anguita M, Torres F, Giménez D, Bergillos F, Ruiz M, Ciudad M, Gallardo A, Vallés F. Incidence of specific etiology and role of methods for specific etiologic diagnosis of primary acute pericarditis. Am J Cardiol. 1995;75:378–82.

    Article  CAS  Google Scholar 

  7. Knockaert DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J. 2007;28:1797–804.

    Article  Google Scholar 

  8. •• Cremer PC, Kumar A, Kontzias A, Tan CD, Rodriguez ER, Imazio M, Klein AL. Complicated pericarditis: understanding risk factors and pathophysiology to inform imaging and treatment. J Am Coll Cardiol. 2016;68:2311–28. This paper focuses on reviewing complicated cases of pericarditis and addresses risk factors, pathophysiology involved, imaging modalities, and novel treatments for such complicated cases.

    Article  Google Scholar 

  9. Xu B, Harb SC, Cremer PC. New insights into pericarditis: mechanisms of injury and therapeutic targets. Curr Cardiol Rep. 2017;19:60.

    Article  Google Scholar 

  10. Park H, Bourla AB, Kastner DL, Colbert RA, Siegel RM. Lighting the fires within: the cell biology of autoinflammatory diseases. Nat Rev Immunol. 2012;12:570–80.

    Article  CAS  Google Scholar 

  11. Booty MG, Chae JJ, Masters SL, Remmers EF, Barham B, Le JM, Barron KS, Holland SM, Kastner DL, Aksentijevich I. Familial Mediterranean fever with a single MEFV mutation: where is the second hit? Arthritis Rheum. 2009;60:1851–61.

    Article  CAS  Google Scholar 

  12. Justiz Vaillant AA, Goyal A, Varacallo M. Systemic lupus erythematosus. StatPearls. 2022.

  13. Man BL, Mok CC. Serositis related to systemic lupus erythematosus: prevalence and outcome. Lupus. 2005;14:822–6.

    Article  CAS  Google Scholar 

  14. Cervera R, Khamashta MA, Font J, et al. Morbidity and mortality in systemic lupus erythematosus during a 10-year period: a comparison of early and late manifestations in a cohort of 1,000 patients. Medicine (Baltimore). 2003;82:299–308.

    Article  Google Scholar 

  15. Ryu S, Fu W, Petri MA. Associates and predictors of pleurisy or pericarditis in SLE. Lupus Sci Med. 2017;4: e000221.

    Article  Google Scholar 

  16. Moder KG, Miller TD, Tazelaar HD. Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc. 1999;74:275–84.

    Article  CAS  Google Scholar 

  17. Kahl LE. The spectrum of pericardial tamponade in systemic lupus erythematosus. Report of ten patients Arthritis Rheum. 1992;35:1343–9.

    Article  CAS  Google Scholar 

  18. Bidani AK, Roberts JL, Schwartz MM, Lewis EJ. Immunopathology of cardiac lesions in fatal systemic lupus erythematosus. Am J Med. 1980;69:849–58.

    Article  CAS  Google Scholar 

  19. Mellana WM, Aronow WS, Palaniswamy C, Khera S. Rheumatoid arthritis: cardiovascular manifestations, pathogenesis, and therapy. Curr Pharm Des. 2012;18:1450–6.

    Article  CAS  Google Scholar 

  20. Voskuyl AE. The heart and cardiovascular manifestations in rheumatoid arthritis. Rheumatology (Oxford). 2006;45:Suppl 4:iv4–7.

  21. Byers RJ, Marshall DA, Freemont AJ. Pericardial involvement in systemic sclerosis. Ann Rheum Dis. 1997;56:393–4.

    Article  CAS  Google Scholar 

  22. Cutolo M, Soldano S, Smith V. Pathophysiology of systemic sclerosis: current understanding and new insights. Expert Rev Clin Immunol. 2019;15:753–64.

    Article  CAS  Google Scholar 

  23. Agard C, Rendu E, Leguern V, Ponge T, Masseau A, Barrier JH, Trochu JN, Hamidou MA, Guillevin L. Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature. Semin Arthritis Rheum. 2007;36:386–91.

    Article  CAS  Google Scholar 

  24. Cullen S, Duff DF, Denham B, Ward OC. Cardiovascular manifestations in Kawasaki disease. Ir J Med Sci. 1989;158:253–6.

    Article  CAS  Google Scholar 

  25. Imazio M, Hoit BD. Post-cardiac injury syndromes. An emerging cause of pericardial diseases. Int J Cardiol. 2013;168:648–52.

    Article  Google Scholar 

  26. Verma BR, Chetrit M, Gentry Iii JL, et al. Multimodality imaging in patients with post-cardiac injury syndrome. Heart. 2020;106:639–46.

    Article  CAS  Google Scholar 

  27. Hoffman M, Fried M, Jabareen F, Vardinon N, Turner D, Burke M, Yust I. Anti-heart antibodies in postpericardiotomy syndrome: cause or epiphenomenon? Autoimmunity. 2002;35:241–5.

    Article  CAS  Google Scholar 

  28. Chahine J, Ala CK, Gentry JL, Pantalone KM, Klein AL. Pericardial diseases in patients with hypothyroidism. Heart. 2019;105:1027–33.

    Article  CAS  Google Scholar 

  29. Hardisty CA, Naik DR, Munro DS. Pericardial effusion in hypothyroidism Clin Endocrinol (Oxf). 1980;13:349–54.

    Article  CAS  Google Scholar 

  30. Nouh MS, Famuyiwa OO, Sulimani RA, Al-Nuaim A. The spectrum of echocardiographic abnormalities in hypothyroidism and the effect of hormonal treatment. Ann Saudi Med. 1991;11:611–5.

    Article  CAS  Google Scholar 

  31. Bajaj R, Mehrzad R, Singh K, Gupta JP. Cardiac tamponade in hypothyroidism. Case Rep. 2014:bcr2014204076.

  32. Schwier NC, O’Neal K. Pharmacotherapeutic management strategies for thyroid disease-induced pericarditis. Ann Pharmacother. 2020;54(5):486–95.

    Article  Google Scholar 

  33. Chahine J, Jedeon Z, Chang KY, Jellis CL. Pericardial manifestations of thyroid diseases. Curr Cardiol Rep. 2022;24(8):893–904. https://doi.org/10.1007/s11886-022-01711-7.

    Article  Google Scholar 

  34. LeWinter M, Cremer PC, Klein AL. Pericardial disease. Braunwald’s heart disease: a textbook of cardiovascular medicine. 2022;2.

  35. Imazio M, Brucato A, Barbieri A, et al. Good prognosis for pericarditis with and without myocardial involvement: results from a multicenter, prospective cohort study. Circulation. 2013;128:42–9.

    Article  CAS  Google Scholar 

  36. Imazio M, Brucato A, Maestroni S, Cumetti D, Dominelli A, Natale G, Trinchero R. Prevalence of C-reactive protein elevation and time course of normalization in acute pericarditis: implications for the diagnosis, therapy, and prognosis of pericarditis. Circulation. 2011;123:1092–7.

    Article  CAS  Google Scholar 

  37. Kumar A, Sato K, Yzeiraj E, Betancor J, Lin L, Tamarappoo BK, Kwon DH, Hachamovitch R, Klein AL. Quantitative pericardial delayed hyperenhancement informs clinical course in recurrent pericarditis. JACC Cardiovasc Imaging. 2017;10:1337–46.

    Article  Google Scholar 

  38. • Klein AL, Abbara S, Agler DA, et al. American Society of Echocardiography clinical recommendations for multimodality cardiovascular imaging of patients with pericardial disease: endorsed by the society for cardiovascular magnetic resonance and society of cardiovascular computed tomography. J Am Soc Echocardiogr. 2013;26:965-1012.e15. This reference is the official clinical recommendations set forth by the American Society of Echocardiography detailing the role of surface echocardiography in the diagnosis and evaluation of pericardial diseases. In addition to echocardiographic information, this reference details the role of CT and/or MRI in further evaluation of pericardial disease should additional images be needed.

  39. Fadl SA, Nasrullah A, Harris A, Edwards R, Kicska G. Comprehensive review of pericardial diseases using different imaging modalities. Int J Cardiovasc Imaging. 2020;36:947–69.

    Article  Google Scholar 

  40. Kearns MJ, Walley KR. Tamponade: hemodynamic and echocardiographic diagnosis. Chest. 2018;153:1266–75.

    Article  Google Scholar 

  41. • Jain V, Chhabra G, Chetrit M, et al. Role of non-invasive multimodality imaging in autoimmune pericarditis. Eur Heart J Cardiovasc Imaging. 2021;22:1228–40. This reference provides a detailed summary of the anatomy and pathophysiology related to autoimmune/autoinflammatory pericardial diseases. In addition, it provides several approaches to using multimodality cardiac imaging in the evaluation of autoimmune pericardial disease, which we expand upon in our review article.

  42. Wang ZJ, Reddy GP, Gotway MB, Yeh BM, Hetts SW, Higgins CB. CT and MR imaging of pericardial disease. Radiographics. 2003;23:Spec No:S167–180.

  43. Manda YR, Baradhi KM. Myopericarditis. StatPearls. 2002.

  44. Hammer MM, Raptis CA, Javidan-Nejad C, Bhalla S. Accuracy of computed tomography findings in acute pericarditis. Acta Radiol. 2014;55:1197–202.

    Article  Google Scholar 

  45. Bogaert J, Francone M. Cardiovascular magnetic resonance in pericardial diseases. J Cardiovasc Magn Reson. 2009;11:14.

    Article  Google Scholar 

  46. Young PM, Glockner JF, Williamson EE, Morris MF, Araoz PA, Julsrud PR, Schaff HV, Edwards WD, Oh JK, Breen JF. MR imaging findings in 76 consecutive surgically proven cases of pericardial disease with CT and pathologic correlation. Int J Cardiovasc Imaging. 2012;28:1099–109.

    Article  Google Scholar 

  47. James OG, Christensen JD, Wong TZ, Borges-Neto S, Koweek LM. Utility of FDG PET/CT in inflammatory cardiovascular disease. Radiographics. 2011;31:1271–86.

    Article  Google Scholar 

  48. Alter P, Figiel JH, Rupp TP, Bachmann GF, Maisch B, Rominger MB. MR, CT, and PET imaging in pericardial disease. Heart Fail Rev. 2013;18:289–306.

    Article  CAS  Google Scholar 

  49. Hyeon CW, Yi HK, Kim EK, Park S-J, Lee S-C, Park SW, Oh JK, Choi JY, Chang S-A. The role of 18F-fluorodeoxyglucose-positron emission tomography/computed tomography in the differential diagnosis of pericardial disease. Sci Rep. 2020;10:21524.

    Article  CAS  Google Scholar 

  50. Imazio M, Adler Y. Treatment with aspirin, NSAID, corticosteroids, and colchicine in acute and recurrent pericarditis. Heart Fail Rev. 2013;18:355–60.

    Article  CAS  Google Scholar 

  51. Alabed S, Cabello JB, Irving GJ, Qintar M, Burls A. Colchicine for pericarditis. Cochrane Database Syst Rev. 2014;CD010652.

  52. Griffin BP. Manual of Cardiovascular Medicine. 2019;5.

  53. Imazio M, Brucato A, Cumetti D, et al. Corticosteroids for recurrent pericarditis: high versus low doses: a nonrandomized observation. Circulation. 2008;118:667–71.

    Article  CAS  Google Scholar 

  54. Vianello F, Cinetto F, Cavraro M, Battisti A, Castelli M, Imbergamo S, Marcolongo R. Azathioprine in isolated recurrent pericarditis: a single centre experience. Int J Cardiol. 2011;147:477–8.

    Article  Google Scholar 

  55. Imazio M, Lazaros G, Picardi E, Vasileiou P, Carraro M, Tousoulis D, Belli R, Gaita F. Intravenous human immunoglobulins for refractory recurrent pericarditis: a systematic review of all published cases. J Cardiovasc Med (Hagerstown). 2016;17:263–9.

    Article  CAS  Google Scholar 

  56. Moretti M, Buiatti A, Merlo M, Massa L, Fabris E, Pinamonti B, Sinagra G. Usefulness of high-dose intravenous human immunoglobulins treatment for refractory recurrent pericarditis. Am J Cardiol. 2013;112:1493–8.

    Article  CAS  Google Scholar 

  57. Lazaros G, Imazio M, Brucato A, Vassilopoulos D, Vasileiou P, Gattorno M, Tousoulis D, Martini A. Anakinra: an emerging option for refractory idiopathic recurrent pericarditis: a systematic review of published evidence. J Cardiovasc Med (Hagerstown). 2016;17:256–62.

    Article  CAS  Google Scholar 

  58. Klein AL, Imazio M, Cremer P, et al. Phase 3 trial of interleukin-1 trap rilonacept in recurrent pericarditis. N Engl J Med. 2021;384:31–41.

    Article  CAS  Google Scholar 

  59. Tombetti E, Mulè A, Tamanini S, Matteucci L, Negro E, Brucato A, Carnovale C. Novel pharmacotherapies for recurrent pericarditis: current options in 2020. Curr Cardiol Rep. 2020;22:59.

    Article  Google Scholar 

  60. Cantarini L, Imazio M, Brizi MG, Lucherini OM, Brucato A, Cimaz R, Galeazzi M. Role of autoimmunity and autoinflammation in the pathogenesis of idiopathic recurrent pericarditis. Clin Rev Allergy Immunol. 2013;44:6–13.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Center for the Diagnosis and Treatment of Pericardial Diseases, Section of Cardiovascular Imaging, Department of Cardiovascular Medicine, Heart, Vascular, and Thoracic Institute, Cleveland Clinic, 9500 Euclid Ave., Desk J1-5, Cleveland, OH, 44195, USA

    Ghazaleh Goldar, Cassandra Garraud, Heba Wassif, Vardhmaan Jain & Allan L. Klein

  2. University of Michigan Department of Internal Medicine, Ann Arbor, MI, USA

    Aaron A. Sifuentes

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  1. Ghazaleh Goldar
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  2. Cassandra Garraud
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  3. Aaron A. Sifuentes
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  5. Vardhmaan Jain
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Correspondence to Ghazaleh Goldar.

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Conflict of Interest

Allan Klein reports research grant from Kiniksa; consulting fees from Cardiol Therapeutics, Pfizer; and honoraria from Kiniksa Pharma for participation on a Data Safety Monitoring Board or Advisory Board. The other authors declare that they have no conflict of interest.

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Ghazaleh Goldar and Cassandra Garraud are first authors.

Vardhmaan Jain and Allan L. Klein are primary investigators.

This article is part of the Topical Collection on Pericardial Disease

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Goldar, G., Garraud, C., Sifuentes, A.A. et al. Autoimmune Pericarditis: Multimodality Imaging. Curr Cardiol Rep 24, 1633–1645 (2022). https://doi.org/10.1007/s11886-022-01785-3

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