Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis: A Meta-analysis of 62 Case Reports
Abstract
Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-systemic vasculitis, with cardiac involvement being one of its most serious manifestations. We aimed to systematically review and analyze the limited case reports of EGPA with cardiac involvement.
Methods: Based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, we performed a systematic literature search for the case reports of EGPA with cardiac involvement in the MEDLINE database from 2011 until 2018. For each case, clinical data including sex, age, clinical presentation, electrocardiographic and cardiac imaging findings, the type of cardiac involvement, the available laboratory data (cardiac biomarkers, white blood cell count, eosinophilic count, erythrocyte sedimentation rate, C-reactive protein, and antineutrophil cytoplasmic antibody positivity), therapeutic regimen, and the outcome of the patients were collected and analyzed.
Results: A total number of 62 cases were included. The mean age was 48.29±15.60 years, and 51.6% were male. All the cases were in the active disease state. Cardiac symptoms, electrocardiographic abnormalities, abnormal biomarkers, and abnormal echocardiography were detected in 82.3%, 68.5%, 77.4%, and 96.8%, respectively. Cardiac magnetic resonance was done in 46.8% of the patients, and it was abnormal in all. The most common abnormal findings in echocardiography were systolic left ventricular dysfunction (83.9%) and pericardial effusion (37.1%). The most common type of clinical presentation was clinical heart failure (51.6%). Only 6.5% of the patients presented with tamponade. The overall prognosis was good.
Conclusion: Any part of the heart could be involved by EGPA. The results emphasize the necessity of in-depth cardiac evaluation in these patients.
Knockaert DC. Cardiac involvement in systemic inflammatory disease. European heart journal 2007;28:1797-1804.
Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301.
Noth I, Strek ME, Leff AR. Churg-Strauss syndrome. Lancet 2003;361:587–594.
Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churge-Strauss syndrome. Clinical study and long-term follow-up of 96 patient. Medicine 1999;78:26-37
Greco A, Rizzo MI, De Virgilio A, Gallo A, Fusconi M, Ruoppolo G, Altissimi G, De Vincentiis M. Churg-Strauss syndrome. Autoimmun Rev 2015 Apr;14:341-348
Pagnoux C, Guillevin L. Peripheral neuropathy in systemic vasculitides. Curr Opin Rheumatol 2005; 17 : 41-48 .
Çiledağ A, Deniz H, Eledağ S, Özkal C, Düzgün N, Erekul S, Karnak D. An aggressive and lethal course of Churg-Strauss syndrome with alveolar hemorrhage, intestinal perforation, cardiac failure and peripheral neuropathy. Rheumatol Int 2012 Feb;32:451-455
Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 1990;33:1094-1100.
Guillevin L, Lhote F, Gayraud M, Cohen P, Jarrousse B, Lortholary O, Thibult N, Casassus P. Prognostic factors in polyarteritis nodosa and Churg–Strauss Syndrome. A prospective study in 342 patients. Medicine 1996;75:17–28
Cohen P, Pagnoux C, Mahr A, Arene JP, Mouthon L, Le Guern V, André MH, Gayraud M, Jayne D, Blöckmans D, Cordier JF, Guillevin. Churg–Strauss syndrome with poor-prognosis factors: a prospective multicenter trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in forty-eight patients. Arthritis Rheum 2007;57:686–693.
Ribi C, Cohen P, Pagnoux C, Mahr A, Arene JP, Lauque D, Puéchal X, Letellier P, Delaval P, Cordier JF, Guillevin L. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum 2008;58:586–94
Yılmaz İ, Tutar N, Şimşek ZÖ, Oymak FS, Gülmez İ. Clinical and serological features of eosinophilic and vasculitic phases of eosinophilic granulomatosis with poliangiitis: a case series of 15 patients. Turkish Thoracic Journal 2017;18:72-77
Zardkoohi O, Hobbs R, Tan CD. A rare shock. Am J Med 2011 ;124:1019-1022
Hazebroek MR, van Paassen P, Dennert R, Heymans S. ANCA-associated vasculitis: microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg–Strauss Syndrome) and granulomatosis With polyangiitis (Wegener’s Granulomatosis). In: Nussinovitch U, ed.The Heart in Rheumatic, Autoimmune and Inflammatory Disease: Pathophysiologic, Clinical Aspect and Therapeutic Approaches. 1rd ed. Academic Press; 2017. p.459-502
Mavrogeni S, Karabela G, Gialafos E, Stavropoulos E, Spiliotis G, Katsifis G, Kolovou G. Cardiac involvement in ANCA (+) and ANCA (-) Churg-Strauss syndrome evaluated by cardiovascular magnetic resonance. Inflamm Allergy Drug Targets 2013;12:322-327.
Szczeklik W, Miszalski-Jamka T. Cardiac involvement in eosinophilic granulomatosis with polyangitis (Churg Strauss). Journal of Rare Cardiovascular Diseases 2013; 1:91-95
Misra DP, Shenoy SN . Cardiac involvement in primary systemic vasculitis and potential drug therapies to reduce cardiovascular risk. Rheumatol Int 2017;37:151–167.
Dennert RM, van Paassen P, Schalla S, Kuznetsova T, Alzand BS, Staessen JA, Velthuis S, Crijns HJ, Tervaert JW, Heymans S. Cardiac involvement in Churg–Strauss syndrome. Arthritis Rheum 2010;62:627–634.
Bourgarit A, Le Toumelin P, Pagnoux C, Cohen P, Mahr A, Le Guern V, Mouthon L, Guillevin L. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg‑Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine 2005;84:323-330.
Kobayashi D, Wada Y, Takata T, Koya T, Murakami S, Ozawa T, Aizawa Y, Kuroda T, Nakano M, Narita I. A severe form of Churg-Strauss syndrome complicated with acute cardiac failure and rapidly progressive peripheral neuropathy--a possible effect of intravenous immunoglobulin therapy. Intern Med 2011;50:925-929
Kakouros N, Bastiaenen R, Kourliouros A, Anderson L. Churg-Strauss presenting as acute coronary syndrome: sometimes it's zebras. BMJ Case Rep. 2011, http://dx.doi.org/10.1136/bcr.01.2011.3703.
Moradinejad MH, Rezaei A, Ziaee V. Juvenile churg-strauss syndrome as an etiology of myocarditis and ischemic stroke in adolescents; a case report. Iran J Pediatr 2011;21:530-534.
Suganuma K, Hashimoto T, Sato H, Suzuki T, Sakurai S. Oculomotor Nerve Palsy following Cardiac Tamponade with Churg-Strauss Syndrome: A Case Report. Case Rep Neurol 2011;3:274-277
Cura L, Dayan V, Cristar F, Soca G. Cardiac denervation procedure to treat refractory angina in a patient with Churg-Strauss syndrome and non-obstructive coronary lesions. Interact Cardiovasc Thorac Surg. 2012;15:166-168
Rigamonti F, De Benedetti E, Letovanec I, Rosset A, Chizzolini C. Cardiac involvement in Churg-Strauss syndrome mimicking acute coronary syndrome. Swiss Med Wklyn 2012; 19;142:w13543
Courand PY, Croisille P, Khouatra C, Cottin V, Kirkorian G, Bonnefoy E. Churg-Strauss syndrome presenting with acute myocarditis and cardiogenic shock. Heart Lung Circ 2012;21:178-81
Gräni C, Langenegger T, Fäh A, Kurz D, Zbinden R, Ramsay D. Perimyocarditis and myocardial infarction A rare manifestation of Churg-Strauss syndrome. Exp Clin Cardiol 2012;17:245-7.
McAleavey N, Millar A, Pendleton A. Cardiac involvement as the main presenting feature in eosinophilic granulomatosis with polyangiitis. BMJ Case Rep 2013; Jul 12;2013.
Correia AS, Gonçalves A, Araújo V, Almeida e Silva J, Pereira JM, Rodrigues Pereira P, Pizarro M, Silva JC, Maciel MJ. Churg-Strauss syndrome presenting with eosinophilic myocarditis: a diagnostic challenge. Rev Port Cardiol 2013;32:707-711.
Isawa Y, Osada S, Omi T, Kawana S. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss Syndrome) with microscopic eosinophilic thromboembolism and cardiac involvement: report of two cases. Eur J Dermatol 2013;23:677-680.
Hara T, Yamaguchi K, Iwase T, Kadota M, Bando M, Ogasawara K, Bando S, Ise T, Niki T, Ueda Y, Tomita N, Taketani Y, Yamada H, Soeki T, Wakatsuki T, Sata M. Eosinophilic myocarditis due to Churg-Strauss syndrome with markedly elevated eosinophil cationic protein. Int Heart J 2013;54:51-53.
Ungprasert P, Srivali N, Cheungpasitporn W, Schaeffer CS. Is it acute coronary syndrome or Churg-Strauss syndrome?. Am J Emerg Med 2013;31:270.e5-8.
Groh M, Masciocco G, Kirchner E, Kristen A, Pellegrini C, Varnous S, Bortman G, Rosenberg M, Brucato A, Waterworth P, Bonacina E, Facchetti F, Calabrese L, Gregorini G, Scali JJ, Starling R, Frigerio M, D'Armini AM, Guillevin L. Heart transplantation in patients with eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). J Heart Lung Transplant 2014;33:842-850.
Papadimitraki ED, Moyssakis I, Mavrogeni S, Mylona M, Anagnostou D, Merkouris K, Barbetseas J. A Churg–Strauss syndrome patient with myopericardial involvement. Journal of Cardiology Cases 2015;11:52-55
Sulaiman W, Seung OP, Noor SM. Acute myocardial infarction as Eosinophilic granulomatosis with polyangiitis (formerly Churg Strauss syndrome) initial presentation. Rev Bras Reumatol 2014;54:393-396.
Chen MX, Yu BL, Peng DQ, Zhou SH.Eosinophilic myocarditis due to Churg-Strauss syndrome mimicking reversible dilated cardiomyopathy. Heart Lung 2014;43:45-47.
Sharpley FA. Missing the beat: arrhythmia as a presenting feature of eosinophilic granulomatosis with polyangiitis. BMJ Case Rep. 2014 May 8;2014. pii: bcr2013203413.
Bouiller K, Samson M, Eicher JC, Audia S, Berthier S, Leguy V, Humbert O, Martin L, Lorgis L, Cottin Y, Bonnotte B, Lorcerie B. Severe cardiomyopathy revealing antineutrophil cytoplasmic antibodies-negative eosinophilic granulomatosis with polyangiitis. Intern Med J 2014 Sep;44:928-931
Załęska J, Wiatr E, Zych J, Szopiński J, Oniszh K, Kober J, Piotrowska-Kownacka D, Roszkowski-Śliż K. Severe congestive heart failure as the main symptom of eosinophilic granulomatosis and polyangiitis (Churg-Strauss syndrome). Pneumonol Alergol Pol 2014;82:582-589.
Cui N, Su L, Wang H, Long Y, Pang C, Yang F, Liu D. A Case Report of Churg-Strauss Syndrome Presenting With Cardiogenic Shock Treated With Extracorporeal Membrane Oxygenation. Medicine (Baltimore) 2015;94:e1757.
Ammirati E, Cipriani M, Musca F, Bonacina E, Pedrotti P, Roghi A, Astaneh A, Schroeder JW, Nonini S, Russo CF, Oliva F, Frigerio M. A life-threatening presentation of eosinophilic granulomatosis with polyangiitis. J Cardiovasc Med (Hagerstown) 2016;17 Suppl 2:e109-e111.
Diaków Z, Koziatek M, Jancewicz P, Całka A, Napora M, Stompór T. Acute myocarditis with heart failure in the course of eosinophilic granulomatosis with polyangiitis in a patient on maintenance hemodialysis. Pol Arch Med Wewn 2015;125:202-203.
Tyagi N, Maheswaran T, Wimalaratna S. ANCA negative eosinophilic granulomatosis with polyangiitis: sometimes it really IS vasculitis. BMJ Case Rep 2015 Dec 23;2015. pii: bcr2015212511.
Jeong HC, Kim KH, Cho JY, Song JE, Yoon HJ, Seon HJ, Ahn Y, Jeong MH, Cho JG, Park JC. Cardiac involvement of churg-strauss syndrome as a reversible cause of dilated cardiomyopathy. J Cardiovasc Ultrasound 2015;23:40-43.
Yano T, Ishimura S, Furukawa T, Koyama M, Tanaka M, Shimoshige S, Hashimoto A, Miura T. Cardiac tamponade leading to the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome): a case report and review of the literature. Heart Vessels 2015;30:841-844.
Hemmett J, Qirjazi E, Weir MA, Moussa M, Landry YD, Gunaratnam L. Cardiac, renal, and central nervous system dysfunction with eosinophilia: eosinophilic granulomatosis with polyangiitis. Lancet 2015;385:480.
Bouabdallaoui N, Arlet JB, Hagege AA. Cardiogenic shock, asthma, and hypereosinophilia. Am J Emerg Med 2015;33:309.e1-2.
Dendramis G, Paleologo C, Piraino D, Arrotti S, Assennato P. Coronary involvement in Churg-Strauss syndrome. Indian Heart J 2015; 67: 586–588.
Dimitriadis Z, Esdorn H, Horstkotte D, Faber L. Coronary spasms, cardiomyopathy, and Churg-Strauss syndrome : a case report. Herz 2015;40:73-75.
Qiao L, Gao D. A case report and literature review of Churg-Strauss syndrome presenting with myocarditis. Medicine (Baltimore) 2016;95:e5080.
Francis W, Aziz Eid Al Kuwari MA, Ghareep AN, Peyrou J, Szmigielski W. A Rare Right Ventricular Involvement of Eosinophilic Granulomatosis Demonstrated by Cardiac MRI. Pol J Radiol 2016;81:598-601.
Bujak R, Błażejewski J, Banach J, Karasek D, Sinkiewicz W. Churg-Strauss syndrome with endocardial injury, clot formation in heart's chambers, and neurological complications. Kardiol Pol 2016;74:1356.
Masaki N, Issiki A, Kirimura M, Kamiyama T, Sasaki O, Ito H, Maruyama Y, Nishioka T. Echocardiographic Changes in Eosinophilic Endocarditis Induced by Churg-Strauss Syndrome. Intern Med 2016;55:2819-2823.
Tsugu T,Nagatomo Y, Yamada Y, Mahara K, Miura H, Murata M. The significance of early screening with echocardiography in eosinophilic granulomatosis with polyangiitis. J Med Ultrason (2001). 2016;43:527-531.
Bang CL, Porsbjerg CM. Think twice - Diagnostic delay in a patient with acute chest pain. Respir Med Case Rep 2016;19:94-97.
Nadeau PL, Kumar A, O'Connor K, Couture CY, Bourgault C, Dubois M, Sénéchal M. Usefulness of cardiac resonance imaging in Churg-Strauss syndrome. J Cardiovasc Med (Hagerstown) 2016;17 Suppl 2:e233-e234.
Keefe AC, Hymas JC, Emerson LL, Ryan JJ. An atypical presentation of cardiac tamponade and periorbital swelling in a patient with eosinophilic granulomatosis with polyangiitis: a case report. J Med Case Rep 2017;11:271.
Zhu D, Luo Y, Liu X, Zu L. Antiproteinase 3 Positive Eosinophilic Granulomatosis with Polyangiitis Presenting with Heart Failure and Intraventricular Thrombosis. Case Rep Rheumatol 2017;2017:2908185.
Jin X, Ma C, Liu S, Guan Z, Wang Y, Yang J. Cardiac involvements in hypereosinophilia-associated syndrome: Case reports and a little review of the literature. Echocardiography 2017;34:1242-1246.
Liu VY, Quintana-Quezada RA, Buja LM. Eosinophilic endomyocardial disease. Cardiovasc Pathol 2017;27:54-56.
Matsuda S, Yoshida S, Fujiki Y, Satomi H, Takeuchi T, Hirose Y, Makino S, Arawaka S. Eosinophilic granulomatosis with polyangiitis complicated by subarachnoid hemorrhage and coronary vasculitis: a case report and review of the literature. Rheumatol Int 2018;38:689-696.
Mattsson G, Magnusson P. Eosinophilic granulomatosis with polyangiitis: myocardial thickening reversed by corticosteroids. BMC Cardiovasc Disord 2017;17:299.
Bluett R, McDonnell D, O'Dowling C, Vaughan C. Eosinophilic myocarditis as a first presentation of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome). BMJ Case Rep. 2017 Nov 23;2017. pii: bcr-2017-221227.
Glaveckaite S, Valeviciene N, Palionis D, Kontrimaviciute E, Lesinskas E. Heart involvement in Churg-Strauss syndrome. Kardiol Pol 2017;75:184.
Pecoraro A, Crescenzi L, Carucci L, Genovese A, Spadaro G. Heart failure not responsive to standard immunosuppressive therapy is successfully treated with high dose intravenous immunoglobulin therapy in a patient with Eosinophilic Granulomatosis with Polyangiitis (EGPA). Int Immunopharmacol 2017;45:13-15.
Nakayama T, Murai S, Ohte N. A Case of dilated Cardiomyopathy with Eosinophilic Granulomatosis with Polyangiitis in which Active Myocardial Inflammation was Only Detected by Endomyocardial Biopsy. Intern Med. 2018 Apr 27. doi: 10.2169/internalmedicine.0330-17.
Apirami M, Pratheepan JA, Kumanan T, Guruparan M, Selvaratnam G. Cardiogenic Shock: An Unusual Initial Presentation of Churg-Strauss Syndrome. Case Rep Rheumatol.2018 Apr 1;2018:2076837.
d'Ersu E, Ribi C, Monney P, Vincenti G, Schwitter J, Rotman S, Hullin R, Regamey J. Churg-Strauss syndrome with cardiac involvement: case illustration and contribution of CMR in the diagnosis and clinical follow-up. Int J Cardiol 2018;258:321-324.
Rastogi U, Sergie Z, Pinney S, Moss N. Recurrence of eosinophilic granulomatosis with polyangitis after orthotopic heart transplant. Am J Transplant 2018;18:1544-1547.
Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009;23: 355–366.
Durel CA, Berthiller J, Caboni S, Jayne D, Ninet J, Hot A. Long-Term Followup of a Multicenter Cohort of 101 Patients With Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss). Arthritis Care Res (Hoboken) 2016;68:374-387.
Hazebroek MR, Kemna MJ, Schalla S, Sanders-van Wijk S, Gerretsen SC, Dennert R, Merken J, Kuznetsova T, Staessen JA, Brunner-La Rocca HP, van Paassen P, Cohen Tervaert JW, Heymans S. Prevalence and prognostic relevance of cardiac involvement in ANCA-associated vasculitis: eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis. Int J Cardiol 2015;199:170-179.
Val-Bernal FJ, Mayorga M, Garcia-Alberdi E, Pozueta JA. Churg–Strauss syndrome and sudden cardiac death. Cardiovasc Pathol 2003;12:94–97
Neumann T, Manger B, Schmid M, Kroegel C, Hansch A, Kaiser WA, Reinhardt D, Wolf G, Hein G, Mall G, Schett G, Zwerina J. Cardiac involvement in Churg-Strauss syndrome: impact of endomyocarditis. Medicine 2009;88:236-43.
Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, Maurier F, Jouneau S, Bienvenu B, Puéchal X, Aumaître O, Le Guenno G, Le Quellec A, Cevallos R, Fain O, Godeau B, Seror R, Dunogué B, Mahr A, Guilpain P, Cohen P, Aouba A, Mouthon L, Guillevin L; French Vasculitis Study Group. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 2013;65:270-281.
McClelland A, Laurent R, Ward M. Steroid responsive coronary stenosis in Churg-Strauss syndrome. Heart. 2011;97:1114.
| Files | ||
| Issue | Vol 15 No 1 (2020): J Teh Univ Heart Ctr | |
| Section | Original Article(s) | |
| DOI | https://doi.org/10.18502/jthc.v15i1.3334 | |
| Keywords | ||
| Churg-Strauss syndrome Anti-neutrophil cytoplasmic antibody-associated vasculitis Cardiovascular diseases | ||
| Rights and permissions | |
|
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License. |
