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Pericarditis in Systemic Rheumatologic Diseases

  • Pericardial Disease (L Klein and CL Jellis, Section Editors)
  • Published:
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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.

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References

  1. Prasad M, Hermann J, Gabriel SE, Weyand CM, Mulvagh S, Mankad R, et al. Cardiorheumatology: cardiac involvement in systemic rheumatic disease. Nat Rev Cardiol. 2015;12(3):168–76.

    PubMed  Google Scholar 

  2. Imazio M. Pericardial involvement in systemic inflammatory diseases. Heart. 2011;97(22):1882–92.

    PubMed  Google Scholar 

  3. Knockaert DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J. 2007;28(15):1797–804.

    PubMed  Google Scholar 

  4. Spodick DH. Pericardial disease. JAMA. 1997;278(9):704.

    PubMed  CAS  Google Scholar 

  5. Imazio M, Cecchi E, Demichelis B, Ierna S, Demarie D, Ghisio A, et al. Indicators of poor prognosis of acute pericarditis. Circulation. 2007;115(21):2739–44.

    PubMed  Google Scholar 

  6. Turiel M, Peretti R, Mornati D, Delfino L, Atzeni F, Sarzi-Puttini P. The role of echocardiographic techniques in connective tissue diseases. Autoimmun Rev. 2005;4(3):171–7.

    PubMed  Google Scholar 

  7. Imazio M, Brucato A, Derosa FG, Lestuzzi C, Bombana E, Scipione F, et al. Aetiological diagnosis in acute and recurrent pericarditis: when and how. J Cardiovasc Med (Hagerstown). 2009;10(3):217–30.

    Google Scholar 

  8. Lilly LS. Treatment of acute and recurrent idiopathic pericarditis. Circulation. 2013;127(16):1723–6.

    PubMed  Google Scholar 

  9. Cremer PC, Kumar A, Kontzias A, Tan CD, Rodriguez ER, Imazio M, et al. Complicated pericarditis: understanding risk factors and pathophysiology to inform imaging and treatment. J Am Coll Cardiol. 2016;68(21):2311–28.

    PubMed  Google Scholar 

  10. Owlia MB, Mostafavi Pour Manshadi SM, Naderi N. Cardiac manifestations of rheumatological conditions: a narrative review. ISRN Rheumatol. 2012;2012:463620.

    PubMed  PubMed Central  Google Scholar 

  11. Brucato A, Brambilla G. Recurrent idiopathic pericarditis: familial occurrence. Int J Cardiol. 2005;102(3):529.

    PubMed  CAS  Google Scholar 

  12. Perricone C, Katz D, Ciccacci C, Ceccarelli F, Valesini G, Shoenfeld Y, et al. The heart matters: contribution of genetic factors in recurrent pericarditis. Isr Med Assoc J. 2019;21(7):487–90.

    PubMed  Google Scholar 

  13. Imazio M, Brucato A, Doria A, Brambilla G, Ghirardello A, Romito A, et al. Antinuclear antibodies in recurrent idiopathic pericarditis: prevalence and clinical significance. Int J Cardiol. 2009;136(3):289–93.

    PubMed  Google Scholar 

  14. Imazio M, Belli R, Brucato A, Cemin R, Ferrua S, Beqaraj F, et al. Efficacy and safety of colchicine for treatment of multiple recurrences of pericarditis (CORP-2): a multicentre, double-blind, placebo-controlled, randomised trial. Lancet. 2014;383(9936):2232–7.

    PubMed  CAS  Google Scholar 

  15. Adler Y, Charron P, Imazio M, Badano L, Baron-Esquivias G, Bogaert J, et al. ESC guidelines for the diagnosis and management of pericardial diseases. Rev Esp Cardiol (Engl Ed). 2015;68(12):1126.

    Google Scholar 

  16. Imazio M, Brucato A, Cumetti D, Brambilla G, Demichelis B, Ferro S, et al. Corticosteroids for recurrent pericarditis: high versus low doses: a nonrandomized observation. Circulation. 2008;118(6):667–71.

    PubMed  CAS  Google Scholar 

  17. Morel N, Bonjour M, Le Guern V, Le Jeunne C, Mouthon L, Piette JC, et al. Colchicine: a simple and effective treatment for pericarditis in systemic lupus erythematosus? A report of 10 cases. Lupus. 2015;24(14):1479–85.

    PubMed  CAS  Google Scholar 

  18. Brucato A, Imazio M, Gattorno M, Lazaros G, Maestroni S, Carraro M, et al. Effect of Anakinra on recurrent pericarditis among patients with colchicine resistance and corticosteroid dependence: the AIRTRIP randomized clinical trial. JAMA. 2016;316(18):1906–12.

    PubMed  Google Scholar 

  19. Allan Klein DL, Cremer P, Nasir S, Luis SA, Abbate A, Akhtar A, et al. Efficacy and safety of rilonacept in recurrent pericarditis: a multicenter phase 2 clinical trial. Circulation. 2019.

  20. Li X, Qian YQ, Liu N, Mu R, Zuo Y, Wang GC, et al. Survival rate, causes of death, and risk factors in systemic sclerosis: a large cohort study. Clin Rheumatol. 2018;37(11):3051–6.

    PubMed  Google Scholar 

  21. Casal-Dominguez M, Pinal-Fernandez I, Corse AM, Paik J, Albayda J, Casciola-Rosen L, et al. Muscular and extramuscular features of myositis patients with anti-U1-RNP autoantibodies. Neurology. 2019;92(13):e1416–e26.

    PubMed  PubMed Central  CAS  Google Scholar 

  22. Yassi U, Iqbal F, Stevenson HL. IgG4-related sclerosing pericarditis in a young man with recurrent chest pain. Ann Thorac Surg. 2019;108(4):e261–e3.

    PubMed  Google Scholar 

  23. Grunewald J, Grutters JC, Arkema EV, Saketkoo LA, Moller DR, Muller-Quernheim J. Sarcoidosis. Nat Rev Dis Primers. 2019;5(1):45.

    PubMed  Google Scholar 

  24. Hu JR, Florido R, Lipson EJ, Naidoo J, Ardehali R, Tocchetti CG, et al. Cardiovascular toxicities associated with immune checkpoint inhibitors. Cardiovasc Res. 2019;115(5):854–68.

    PubMed  PubMed Central  CAS  Google Scholar 

  25. Buppajamrntham T, Palavutitotai N, Katchamart W. Clinical manifestation, diagnosis, management, and treatment outcome of pericarditis in patients with systemic lupus erythematosus. J Med Assoc Thail. 2014;97(12):1234–40.

    Google Scholar 

  26. Dein E, Douglas H, Petri M, Law G, Timlin H. Pericarditis in lupus. Cureus. 2019;11(3):e4166.

    PubMed  PubMed Central  Google Scholar 

  27. Thomas G, Cohen Aubart F, Chiche L, Haroche J, Hie M, Hervier B, et al. Lupus myocarditis: initial presentation and longterm outcomes in a multicentric series of 29 patients. J Rheumatol. 2017;44(1):24–32.

    PubMed  CAS  Google Scholar 

  28. Moder KG, Miller TD, Tazelaar HD. Cardiac involvement in systemic lupus erythematosus. Mayo Clin Proc. 1999;74(3):275–84.

    PubMed  CAS  Google Scholar 

  29. Man BL, Mok CC. Serositis related to systemic lupus erythematosus: prevalence and outcome. Lupus. 2005;14(10):822–6.

    PubMed  CAS  Google Scholar 

  30. Ryu S, Fu W, Petri MA. Associates and predictors of pleurisy or pericarditis in SLE. Lupus Sci Med. 2017;4(1):e000221.

    PubMed  PubMed Central  Google Scholar 

  31. Aringer M. Inflammatory markers in systemic lupus erythematosus. J Autoimmun. 2020;110:102374.

    PubMed  CAS  Google Scholar 

  32. 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):iv8–13.

    Google Scholar 

  33. Kahl LE. The spectrum of pericardial tamponade in systemic lupus erythematosus. Report of ten patients. Arthritis Rheum. 1992;35(11):1343–9.

    PubMed  CAS  Google Scholar 

  34. Quismorio FP Jr. Immune complexes in the pericardial fluid in systemic lupus erythematosus. Arch Intern Med. 1980;140(1):112–4.

    PubMed  Google Scholar 

  35. Bidani AK, Roberts JL, Schwartz MM, Lewis EJ. Immunopathology of cardiac lesions in fatal systemic lupus erythematosus. Am J Med. 1980;69(6):849–58.

    PubMed  CAS  Google Scholar 

  36. Celhar T, Fairhurst AM. Modelling clinical systemic lupus erythematosus: similarities, differences and success stories. Rheumatology (Oxford). 2007;56(suppl_1):i88–99.

    Google Scholar 

  37. Stehbens WE, Zuccollo JM. Anitschkow myocytes or cardiac histiocytes in human hearts. Pathology. 1999;31(2):98–101.

    PubMed  CAS  Google Scholar 

  38. Doria A, Iaccarino L, Sarzi-Puttini P, Atzeni F, Turriel M, Petri M. Cardiac involvement in systemic lupus erythematosus. Lupus. 2005;14(9):683–6.

    PubMed  CAS  Google Scholar 

  39. Fanouriakis A, Kostopoulou M, Alunno A, Aringer M, Bajema I, Boletis JN, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–45.

    PubMed  CAS  Google Scholar 

  40. Corrao S, Messina S, Pistone G, Calvo L, Scaglione R, Licata G. Heart involvement in rheumatoid arthritis: systematic review and meta-analysis. Int J Cardiol. 2013;167(5):2031–8.

    PubMed  Google Scholar 

  41. Gordon DA, Stein JL, Broder I. The extra-articular features of rheumatoid arthritis. A systematic analysis of 127 cases. Am J Med. 1973;54(4):445–52.

    PubMed  CAS  Google Scholar 

  42. Hara KS, Ballard DJ, Ilstrup DM, Connolly DC, Vollertsen RS. Rheumatoid pericarditis: clinical features and survival. Medicine (Baltimore). 1990;69(2):81–91.

    CAS  Google Scholar 

  43. Jurik AG, Graudal H. Pericarditis in rheumatoid arthritis. A clinical and radiological study. Rheumatol Int. 1986;6(1):37–42.

    PubMed  CAS  Google Scholar 

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

    Google Scholar 

  45. McRorie ER, Wright RA, Errington ML, Luqmani RA. Rheumatoid constrictive pericarditis. Br J Rheumatol. 1997;36(1):100–3.

    PubMed  CAS  Google Scholar 

  46. Edwards MH, Leak AM. Pericardial effusions on anti-TNF therapy for rheumatoid arthritis--a drug side effect or uncontrolled systemic disease? Rheumatology (Oxford). 2009;48(3):316–7.

    CAS  Google Scholar 

  47. Cauduro SA, Moder KG, Luthra HS, Seward JB. Echocardiographically guided pericardiocentesis for treatment of clinically significant pericardial effusion in rheumatoid arthritis. J Rheumatol. 2006;33(11):2173–7.

    PubMed  Google Scholar 

  48. Soh MC, Hart HH, Corkill M. Pericardial effusions with tamponade and visceral constriction in patients with rheumatoid arthritis on tumour necrosis factor (TNF)-inhibitor therapy. Int J Rheum Dis. 2009;12(1):74–7.

    PubMed  Google Scholar 

  49. Murashita T, Schaff HV, Daly RC, Oh JK, Dearani JA, Stulak JM, et al. Experience with pericardiectomy for constrictive pericarditis over eight decades. Ann Thorac Surg. 2017;104(3):742–50.

    PubMed  Google Scholar 

  50. Butt SA, Jeppesen JL, Torp-Pedersen C, Sam F, Gislason GH, Jacobsen S, et al. Cardiovascular manifestations of systemic sclerosis: a Danish nationwide cohort study. J Am Heart Assoc. 2019;8(17):e013405.

    PubMed  PubMed Central  Google Scholar 

  51. Fernandez Morales A, Iniesta N, Fernandez-Codina A, Vaz de Cunha J, Perez Romero T, Hurtado Garcia R, et al. Cardiac tamponade and severe pericardial effusion in systemic sclerosis: report of nine patients and review of the literature. Int J Rheum Dis. 2017;20(10):1582–92.

    PubMed  Google Scholar 

  52. Follansbee WP, Zerbe TR, Medsger TA Jr. Cardiac and skeletal muscle disease in systemic sclerosis (scleroderma): a high risk association. Am Heart J. 1993;125(1):194–203.

    PubMed  CAS  Google Scholar 

  53. Mukherjee M, Chung SE, Ton VK, Tedford RJ, Hummers LK, Wigley FM, et al. Unique abnormalities in right ventricular longitudinal strain in systemic sclerosis patients. Circ Cardiovasc Imaging. 2016;9(6).

  54. Sagrista-Sauleda J, Merce J, Permanyer-Miralda G, Soler-Soler J. Clinical clues to the causes of large pericardial effusions. Am J Med. 2000;109(2):95–101.

    PubMed  CAS  Google Scholar 

  55. Pokeerbux MR, Giovannelli J, Dauchet L, Mouthon L, Agard C, Lega JC, et al. Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature. Arthritis Res Ther. 2019;21(1):86.

    PubMed  PubMed Central  CAS  Google Scholar 

  56. Ioannidis JP, Vlachoyiannopoulos PG, Haidich AB, Medsger TA Jr, Lucas M, Michet CJ, et al. Mortality in systemic sclerosis: an international meta-analysis of individual patient data. Am J Med. 2005;118(1):2–10.

    PubMed  Google Scholar 

  57. Byers RJ, Marshall DA, Freemont AJ. Pericardial involvement in systemic sclerosis. Ann Rheum Dis. 1997;56(6):393–4.

    PubMed  PubMed Central  CAS  Google Scholar 

  58. Thompson AE, Pope JE. A study of the frequency of pericardial and pleural effusions in scleroderma. Br J Rheumatol. 1998;37(12):1320–3.

    PubMed  CAS  Google Scholar 

  59. Simeon-Aznar CP, Fonollosa-Pla V, Tolosa-Vilella C, Espinosa-Garriga G, Ramos-Casals M, Campillo-Grau M, et al. Registry of the Spanish network for systemic sclerosis: clinical pattern according to cutaneous subsets and immunological status. Semin Arthritis Rheum. 2012;41(6):789–800.

    PubMed  Google Scholar 

  60. Cutolo M, Soldano S, Smith V. Pathophysiology of systemic sclerosis: current understanding and new insights. Expert Rev Clin Immunol. 2019;15(7):753–64.

    PubMed  CAS  Google Scholar 

  61. Kitchongcharoenying P, Foocharoen C, Mahakkanukrauh A, Suwannaroj S, Nanagara R. Pericardial fluid profiles of pericardial effusion in systemic sclerosis patients. Asian Pac J Allergy Immunol. 2013;31(4):314–9.

    PubMed  Google Scholar 

  62. Deswal A, Follansbee WP. Cardiac involvement in scleroderma. Rheum Dis Clin N Am. 1996;22(4):841–60.

    CAS  Google Scholar 

  63. Ferri C, Giuggioli D, Sebastiani M, Colaci M, Emdin M. Heart involvement and systemic sclerosis. Lupus. 2005;14(9):702–7.

    PubMed  CAS  Google Scholar 

  64. Kowal-Bielecka O, Fransen J, Avouac J, Becker M, Kulak A, Allanore Y, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017;76(8):1327–39.

    PubMed  Google Scholar 

  65. Fernandez-Codina A, Walker KM, Pope JE, Scleroderma AG. Treatment algorithms for systemic sclerosis according to experts. Arthritis Rheum. 2018;70(11):1820–8.

    CAS  Google Scholar 

  66. Dunne JV, Chou JP, Viswanathan M, Wilcox P, Huang SH. Cardiac tamponade and large pericardial effusions in systemic sclerosis: a report of four cases and a review of the literature. Clin Rheumatol. 2011;30(3):433–8.

    PubMed  Google Scholar 

  67. Okereke Q, Sutcliffe S, Hanson J. Constrictive, calcific pericarditis in a scleroderma patient. J Clin Rheumatol. 2017;23(7):393.

    PubMed  Google Scholar 

  68. Parks JL, Taylor MH, Parks LP, Silver RM. Systemic sclerosis and the heart. Rheum Dis Clin N Am. 2014;40(1):87–102.

    Google Scholar 

  69. Miloslavsky E, Unizony S. The heart in vasculitis. Rheum Dis Clin N Am. 2014;40(1):11–26.

    Google Scholar 

  70. Fateh-Moghadam S, Huehns S, Schmidt WA, Dietz R, Bocksch W. Pericardial effusion as primary manifestation of Takayasu arteritis. Int J Cardiol. 2010;145(1):e33–5.

    PubMed  Google Scholar 

  71. Maksimowicz-McKinnon K, Clark TM, Hoffman GS. Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients. Arthritis Rheum. 2007;56(3):1000–9.

    PubMed  Google Scholar 

  72. Zenone T, Puget M. Pericardial effusion and giant cell arteritis. Rheumatol Int. 2014;34(10):1465–9.

    PubMed  Google Scholar 

  73. Matsue Y, Ohno M, Nagahori W, Suzuki M, Matsumura A, Hashimoto Y. A case of giant cell arteritis with massive pericardial effusion. Heart Vessel. 2011;26(5):562–4.

    Google Scholar 

  74. Tiosano S, Adler Y, Azrielant S, Yavne Y, Gendelman O, Ben-Ami Shor D, et al. Pericarditis among giant cell arteritis patients: from myth to reality. Clin Cardiol. 2018;41(5):623–7.

    PubMed  PubMed Central  Google Scholar 

  75. Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616–26.

    PubMed  Google Scholar 

  76. Dennert RM, van Paassen P, Schalla S, Kuznetsova T, Alzand BS, Staessen JA, et al. Cardiac involvement in Churg-Strauss syndrome. Arthritis Rheum. 2010;62(2):627–34.

    PubMed  Google Scholar 

  77. Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52(9):2926–35.

    PubMed  CAS  Google Scholar 

  78. Forstot JZ, Overlie PA, Neufeld GK, Harmon CE, Forstot SL. Cardiac complications of Wegener granulomatosis: a case report of complete heart block and review of the literature. Semin Arthritis Rheum. 1980;10(2):148–54.

    PubMed  CAS  Google Scholar 

  79. Geri G, Wechsler B, Thi Huong d L, Isnard R, Piette JC, Amoura Z, et al. Spectrum of cardiac lesions in Behcet disease: a series of 52 patients and review of the literature. Medicine (Baltimore). 2012;91(1):25–34.

    Google Scholar 

  80. Guillevin L, Dorner T. Vasculitis: mechanisms involved and clinical manifestations. Arthritis Res Ther. 2007;9(Suppl 2):S9.

    PubMed  PubMed Central  Google Scholar 

  81. Dewan R, Trejo Bittar HE, Lacomis J, Ocak I. Granulomatosis with polyangiitis presenting with coronary artery and pericardial involvement. Case Rep Radiol. 2015;2015:516437.

    PubMed  PubMed Central  Google Scholar 

  82. Geetha D, Jin Q, Scott J, Hruskova Z, Hanouneh M, Little MA, et al. Comparisons of guidelines and recommendations on managing antineutrophil cytoplasmic antibody-associated vasculitis. Kidney Int Rep. 2018;3(5):1039–49.

    PubMed  PubMed Central  Google Scholar 

  83. de Jesus AA, Canna SW, Liu Y, Goldbach-Mansky R. Molecular mechanisms in genetically defined autoinflammatory diseases: disorders of amplified danger signaling. Annu Rev Immunol. 2015;33:823–74.

    PubMed  PubMed Central  Google Scholar 

  84. Imazio M, Adler Y, Charron P. Recurrent pericarditis: modern approach in 2016. Curr Cardiol Rep. 2016;18(6):50.

    PubMed  Google Scholar 

  85. Kontzias A, Efthimiou P. Adult-onset Still’s disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs. 2008;68(3):319–37.

    PubMed  CAS  Google Scholar 

  86. Kees S, Langevitz P, Zemer D, Padeh S, Pras M, Livneh A. Attacks of pericarditis as a manifestation of Familial Mediterranean Fever (FMF). QJM. 1997;90(10):643–7.

    PubMed  CAS  Google Scholar 

  87. Zimand S, Tauber T, Hegesch T, Aladjem M. Familial Mediterranean Fever presenting with massive cardiac tamponade. Clin Exp Rheumatol. 1994;12(1):67–9.

    PubMed  CAS  Google Scholar 

  88. Alsarah A, Alsara O, Laird-Fick HS. Cardiac manifestations of Familial Mediterranean fever. Avicenna J Med. 2017;7(4):158–63.

    PubMed  PubMed Central  Google Scholar 

  89. Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, et al. Familial Mediterranean Fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore). 2005;84(1):1–11.

    Google Scholar 

  90. Tutar E, Yalcinkaya F, Ozkaya N, Ekim M, Atalay S. Incidence of pericardial effusion during attacks of Familial Mediterranean Fever. Heart. 2003;89(10):1257–8.

    PubMed  PubMed Central  CAS  Google Scholar 

  91. Heilig R, Broz P. Function and mechanism of the pyrin inflammasome. Eur J Immunol. 2018;48(2):230–8.

    PubMed  CAS  Google Scholar 

  92. Lu A, Wu H. Structural mechanisms of inflammasome assembly. FEBS J. 2015;282(3):435–44.

    PubMed  CAS  Google Scholar 

  93. Gafni J, Ravid M, Sohar E. The role of amyloidosis in Familial Mediterranean Fever. A population study. Isr J Med Sci. 1968;4(5):995–9.

    PubMed  CAS  Google Scholar 

  94. Leung YY, Yao Hui LL, Kraus VB. Colchicine--update on mechanisms of action and therapeutic uses. Semin Arthritis Rheum. 2015;45(3):341–50.

    PubMed  PubMed Central  CAS  Google Scholar 

  95. De Benedetti F, Gattorno M, Anton J, Ben-Chetrit E, Frenkel J, Hoffman HM, et al. Canakinumab for the treatment of autoinflammatory recurrent fever syndromes. N Engl J Med. 2018;378(20):1908–19.

    PubMed  Google Scholar 

  96. Rigante D, Cantarini L, Imazio M, Lucherini OM, Sacco E, Galeazzi M, et al. Autoinflammatory diseases and cardiovascular manifestations. Ann Med. 2011;43(5):341–6.

    PubMed  CAS  Google Scholar 

  97. Bulua AC, Mogul DB, Aksentijevich I, Singh H, He DY, Muenz LR, et al. Efficacy of etanercept in the tumor necrosis factor receptor-associated periodic syndrome: a prospective, open-label, dose-escalation study. Arthritis Rheum. 2012;64(3):908–13.

    PubMed  CAS  Google Scholar 

  98. van der Hilst JC, Simon A, Drenth JP. Hereditary periodic fever and reactive amyloidosis. Clin Exp Med. 2005;5(3):87–98.

    PubMed  Google Scholar 

  99. Camprubi D, Mitjavila F, Arostegui JI, Corbella X. Efficacy of anakinra in an adult patient with recurrent pericarditis and cardiac tamponade as initial manifestations of tumor necrosis factor receptor-associated periodic syndrome due to the R92Q TNFRSF1A variant. Int J Rheum Dis. 2017;20(4):510–4.

    PubMed  CAS  Google Scholar 

  100. Kadavath S, Efthimiou P. Adult-onset Still’s disease-pathogenesis, clinical manifestations, and new treatment options. Ann Med. 2015;47(1):6–14.

    PubMed  CAS  Google Scholar 

  101. Cush JJ, Medsger TA Jr, Christy WC, Herbert DC, Cooperstein LA. Adult-onset Still’s disease. Clinical course and outcome. Arthritis Rheum. 1987;30(2):186–94.

    PubMed  CAS  Google Scholar 

  102. Saper VE, Chen G, Deutsch GH, Guillerman RP, Birgmeier J, Jagadeesh K, et al. Emergent high fatality lung disease in systemic juvenile arthritis. Ann Rheum Dis. 2019;78(12):1722–31.

    PubMed  CAS  Google Scholar 

  103. Hu QY, Zeng T, Sun CY, Luo CN, Liu S, Ding TT, et al. Clinical features and current treatments of adult-onset Still’s disease: a multicentre survey of 517 patients in China. Clin Exp Rheumatol. 2019;37(Suppl 121(6)):52–7.

    PubMed  Google Scholar 

  104. Dall’Ara F, Frassi M, Tincani A, Airo P. A retrospective study of patients with adult-onset Still’s disease: is pericarditis a possible predictor for biological disease-modifying anti-rheumatic drugs need? Clin Rheumatol. 2016;35(8):2117–23.

    PubMed  Google Scholar 

  105. Jara Calabuig I, Sanchez Soriano RM, Marco Domingo TF, Perez Ortiz C, Chamorro Fernandez AJ, Chamorro Fernandez CI. Recurrent pericarditis as the presenting form of adult Still’s disease. Rev Esp Cardiol (Engl Ed). 2017;70(3):208–9.

    Google Scholar 

  106. Fitzgerald AA, Leclercq SA, Yan A, Homik JE, Dinarello CA. Rapid responses to anakinra in patients with refractory adult-onset Still’s disease. Arthritis Rheum. 2005;52(6):1794–803.

    PubMed  CAS  Google Scholar 

  107. Novak S, Anic F, Luke-Vrbanic TS. Extremely high serum ferritin levels as a main diagnostic tool of adult-onset Still’s disease. Rheumatol Int. 2012;32(4):1091–4.

    PubMed  CAS  Google Scholar 

  108. Yoo DH. Biologics for the treatment of adult-onset Still’s disease. Expert Opin Biol Ther. 2019;19(11):1173–90.

    PubMed  CAS  Google Scholar 

  109. Kawaguchi H, Tsuboi H, Yagishita M, Terasaki T, Terasaki M, Shimizu M, et al. Severe adult-onset still disease with constrictive pericarditis and pleuritis that was successfully treated with tocilizumab in addition to corticosteroids and cyclosporin a. Intern Med. 2018;57(7):1033–8.

    PubMed  Google Scholar 

  110. Waghmare S, Valecka B, Cairns AP. A severe case of adult onset stills disease with myopericarditis, resistant to treatment with tocilizumab but responsive to Anakinra. Ulster Med J. 2015;84(2):130–2.

    PubMed  Google Scholar 

  111. Kotter I, Wacker A, Koch S, Henes J, Richter C, Engel A, et al. Anakinra in patients with treatment-resistant adult-onset Still’s disease: four case reports with serial cytokine measurements and a review of the literature. Semin Arthritis Rheum. 2007;37(3):189–97.

    PubMed  Google Scholar 

  112. • Kougkas N, Fanouriakis A, Papalopoulos I, Bertsias G, Avgoustidis N, Repa A, et al. Canakinumab for recurrent rheumatic disease associated-pericarditis: a case series with long-term follow-up. Rheumatology (Oxford). 2018;57(8):1494–5. This study is one of the first to demonstrate that canakinumab, an interleukin 1β blocker, can be effective in recurrent pericarditis related to rheumatic diseases.

    CAS  Google Scholar 

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Authors and Affiliations

  1. Division of Rheumatology, Allergy and Immunology, Department of Internal Medicine, Stony Brook University School of Medicine, Stony Brook, NY, USA

    Apostolos Kontzias, Amir Barkhodari & QingPing Yao

Authors
  1. Apostolos Kontzias
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  2. Amir Barkhodari
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  3. QingPing Yao
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Contributions

Apostolos Kontzias, Amir Barkhodari, and QingPing Yao have equally contributed to the preparation of the manuscript.

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Correspondence to Apostolos Kontzias.

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Conflict of Interest

Apostolos Kontzias has received honoraria and has been a consultant for Novartis and Kiniksa and has served as an ad hoc the advisory board member for Sobi and Lilly. Amir Barkhodari has nothing to declare. Apostolos Kontzias reports no conflicts of interest. QingPing Yao has no conflicts of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Pericardial Disease

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Kontzias, A., Barkhodari, A. & Yao, Q. Pericarditis in Systemic Rheumatologic Diseases. Curr Cardiol Rep 22, 142 (2020). https://doi.org/10.1007/s11886-020-01415-w

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  • DOI: https://doi.org/10.1007/s11886-020-01415-w

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