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Cardiogenic shock, asthma, and hypereosinophilia
American Journal of Emergency Medicine xxx (2014) xxx–xxx
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Cardiogenic shock, asthma, and hypereosinophilia Abstract Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss) is a multisystem syndrome associating asthma, hypereosinophilia, and signs of peripheral vasculitis. We report the case of a 21-year-old man admitted for cardiogenic shock revealing a severe left and right ventricular dysfunction. Hypereosinophilia, history of asthma, and peripheral neuropathy strongly suggested the diagnosis of EGPA. Cardiac magnetic resonance imaging confirmed heart involvement with a diffuse subendocardial late gadolinium enhancement. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, and inotropes. At 6-month follow-up, the patient is doing well. Cardiac involvement is rare in EGPA and often carries poor prognosis. Specific early steroid therapy may improve hemodynamic at short term, allowing postponing the need for circulatory mechanical support or heart transplantation. A 21-year-old male patient was referred to our intensive care unit for cardiogenic shock. Transthoracic echocardiography displayed severe left ventricular (LV) and right ventricular (RV) dysfunctions (Video 1). Brain natriuretic peptide levels rose to 2072 ng/L and leukocyte count to 13 100/mm 3 with 4470/mm 3 (34.1%) eosinophil cells. Cardiac magnetic resonance imaging showed depressed LV and RV ejection fractions (EFs, 15% and 20%, respectively) with extensive biventricular subendocardial late gadolinium enhancement (LGE) and bilateral ventricular thrombi (Fig. 1A-C; Video 2). The patient was diagnosed with asthma 4 years ago and suffered for 1 month from
progressive painful mononeuropathy of the right peroneal nerve proved by electromyography. The association of asthma, hypereosinophilia, peripheral axonal neuropathy, and heart failure (HF) led to the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss). Antineutrophil cytoplasmic antibodies (ANCAs) were negative. Patient's condition rapidly improved with high doses of corticosteroids (15 mg/kg per day for 3 days followed by 1 mg/kg per day) associated to intravenous cyclophosphamide (0.6 g/m2 at days 0, 15, and 30 followed by 0.7 g/m2 every 4 weeks for 4 months), allowing weaning inotropic support within 1 week. Cardiac magnetic resonance imaging performed at 1 month showed an improvement in LVEF (35%) and RVEF (37%), along with the disappearance of cardiac thrombi (Fig. 2 A-C; Video 3). At 6-month follow-up, the patient is doing well with a complete relief of neuropathic pain; LVEF is at 40% on transthoracic echocardiography (Video 4). The diagnosis of EGPA should be evoked in acute HF particularly when associated with recent asthma and hypereosinophilia, whereas histologic evidence of necrotizing vasculitis on tissue biopsies may be conclusive. The prevalence of this vasculitis is 7 to 13 cases per million inhabitants [1]. Circulating ANCAs with specificity for myeloperoxidase are of great interest for the diagnosis but are negative in almost 50% of cases. Heart involvement occurs in approximately 8% to 20% of EGPA patients and is more frequent in ANCA-negative patients (almost30% of patients with HF are ANCA positive). It often carries a poor prognosis [2]. Specific early steroid therapy may improve hemodynamic in the short term, allowing postponing the need for circulatory mechanical support or heart transplantation. For patients with refractory forms of EGPA, heart
Fig. 1. Cardiac magnetic resonance imaging at admission with LGE (short-axis view [A], long-axis view [B], and 4-chamber view [C]) showing extensive subendocardial enhancement of both RV and LV associated with multiple intracardiac thrombi (arrows). Left ventricular and RV dimensions are normal.
0735-6757/© 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Bouabdallaoui N, et al, Cardiogenic shock, asthma, and hypereosinophilia, Am J Emerg Med (2014), http://dx.doi.org/ 10.1016/j.ajem.2014.08.019
2
N. Bouabdallaoui et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 2. Cardiac magnetic resonance imaging with LGE sequences at 1 month under steroid therapy (short-axis view [A], long-axis view [B], and 4-chamber-view [C]) showing a marked reduction in LGE of both the LV and RV with a complete disappearance of intracardiac thrombi.
transplantation should be performed. The prognosis of this specific group is uncertain. We have no conflict of interest to disclose with regard to this work. We certify that the final manuscript has been approved by each author, and we have taken great care to ensure the integrity of this work. We also certify that none of the article's contents have been previously published and that neither the article nor any of its part is under concurrent consideration by any other publication. Furthermore, the authors are open to any suggestion or correction from the reviewers or the editors to improve the manuscript.
Jean-Benoît Arlet MD, PhD Sorbonne Paris Cité, University Paris Descartes, Paris, France Department of Internal Medicine, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris, Paris, France Albert A. Hagege MD, PhD Department of Cardiology, Hôpital Européen Georges Pompidou Assistance Publique des Hôpitaux de Paris, Paris, France Sorbonne Paris Cité, University Paris Descartes, Paris, France
Appendix A. Supplementary data http://dx.doi.org/10.1016/j.ajem.2014.08.019 Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ajem.2014.08.019. References Nadia Bouabdallaoui MD Department of Cardiology, Hôpital Européen Georges Pompidou Assistance Publique des Hôpitaux de Paris, Paris, France Sorbonne Paris Cité, University Paris Descartes, Paris, France Corresponding author. Tel.: + 33 1 84 82 71 77 fax: + 33 1 42 16 55 76 E-mail address: [email protected]
[1] Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65(1):1–11. [2] Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. 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(1): 270–81.
Please cite this article as: Bouabdallaoui N, et al, Cardiogenic shock, asthma, and hypereosinophilia, Am J Emerg Med (2014), http://dx.doi.org/ 10.1016/j.ajem.2014.08.019
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Cardiogenic shock, asthma, and hypereosinophilia Abstract Eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss) is a multisystem syndrome associating asthma, hypereosinophilia, and signs of peripheral vasculitis. We report the case of a 21-year-old man admitted for cardiogenic shock revealing a severe left and right ventricular dysfunction. Hypereosinophilia, history of asthma, and peripheral neuropathy strongly suggested the diagnosis of EGPA. Cardiac magnetic resonance imaging confirmed heart involvement with a diffuse subendocardial late gadolinium enhancement. The patient was successfully treated with systemic corticosteroids, intravenous cyclophosphamide, and inotropes. At 6-month follow-up, the patient is doing well. Cardiac involvement is rare in EGPA and often carries poor prognosis. Specific early steroid therapy may improve hemodynamic at short term, allowing postponing the need for circulatory mechanical support or heart transplantation. A 21-year-old male patient was referred to our intensive care unit for cardiogenic shock. Transthoracic echocardiography displayed severe left ventricular (LV) and right ventricular (RV) dysfunctions (Video 1). Brain natriuretic peptide levels rose to 2072 ng/L and leukocyte count to 13 100/mm 3 with 4470/mm 3 (34.1%) eosinophil cells. Cardiac magnetic resonance imaging showed depressed LV and RV ejection fractions (EFs, 15% and 20%, respectively) with extensive biventricular subendocardial late gadolinium enhancement (LGE) and bilateral ventricular thrombi (Fig. 1A-C; Video 2). The patient was diagnosed with asthma 4 years ago and suffered for 1 month from
progressive painful mononeuropathy of the right peroneal nerve proved by electromyography. The association of asthma, hypereosinophilia, peripheral axonal neuropathy, and heart failure (HF) led to the diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) (formerly Churg-Strauss). Antineutrophil cytoplasmic antibodies (ANCAs) were negative. Patient's condition rapidly improved with high doses of corticosteroids (15 mg/kg per day for 3 days followed by 1 mg/kg per day) associated to intravenous cyclophosphamide (0.6 g/m2 at days 0, 15, and 30 followed by 0.7 g/m2 every 4 weeks for 4 months), allowing weaning inotropic support within 1 week. Cardiac magnetic resonance imaging performed at 1 month showed an improvement in LVEF (35%) and RVEF (37%), along with the disappearance of cardiac thrombi (Fig. 2 A-C; Video 3). At 6-month follow-up, the patient is doing well with a complete relief of neuropathic pain; LVEF is at 40% on transthoracic echocardiography (Video 4). The diagnosis of EGPA should be evoked in acute HF particularly when associated with recent asthma and hypereosinophilia, whereas histologic evidence of necrotizing vasculitis on tissue biopsies may be conclusive. The prevalence of this vasculitis is 7 to 13 cases per million inhabitants [1]. Circulating ANCAs with specificity for myeloperoxidase are of great interest for the diagnosis but are negative in almost 50% of cases. Heart involvement occurs in approximately 8% to 20% of EGPA patients and is more frequent in ANCA-negative patients (almost30% of patients with HF are ANCA positive). It often carries a poor prognosis [2]. Specific early steroid therapy may improve hemodynamic in the short term, allowing postponing the need for circulatory mechanical support or heart transplantation. For patients with refractory forms of EGPA, heart
Fig. 1. Cardiac magnetic resonance imaging at admission with LGE (short-axis view [A], long-axis view [B], and 4-chamber view [C]) showing extensive subendocardial enhancement of both RV and LV associated with multiple intracardiac thrombi (arrows). Left ventricular and RV dimensions are normal.
0735-6757/© 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Bouabdallaoui N, et al, Cardiogenic shock, asthma, and hypereosinophilia, Am J Emerg Med (2014), http://dx.doi.org/ 10.1016/j.ajem.2014.08.019
2
N. Bouabdallaoui et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 2. Cardiac magnetic resonance imaging with LGE sequences at 1 month under steroid therapy (short-axis view [A], long-axis view [B], and 4-chamber-view [C]) showing a marked reduction in LGE of both the LV and RV with a complete disappearance of intracardiac thrombi.
transplantation should be performed. The prognosis of this specific group is uncertain. We have no conflict of interest to disclose with regard to this work. We certify that the final manuscript has been approved by each author, and we have taken great care to ensure the integrity of this work. We also certify that none of the article's contents have been previously published and that neither the article nor any of its part is under concurrent consideration by any other publication. Furthermore, the authors are open to any suggestion or correction from the reviewers or the editors to improve the manuscript.
Jean-Benoît Arlet MD, PhD Sorbonne Paris Cité, University Paris Descartes, Paris, France Department of Internal Medicine, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris, Paris, France Albert A. Hagege MD, PhD Department of Cardiology, Hôpital Européen Georges Pompidou Assistance Publique des Hôpitaux de Paris, Paris, France Sorbonne Paris Cité, University Paris Descartes, Paris, France
Appendix A. Supplementary data http://dx.doi.org/10.1016/j.ajem.2014.08.019 Supplementary data to this article can be found online at http://dx. doi.org/10.1016/j.ajem.2014.08.019. References Nadia Bouabdallaoui MD Department of Cardiology, Hôpital Européen Georges Pompidou Assistance Publique des Hôpitaux de Paris, Paris, France Sorbonne Paris Cité, University Paris Descartes, Paris, France Corresponding author. Tel.: + 33 1 84 82 71 77 fax: + 33 1 42 16 55 76 E-mail address: [email protected]
[1] Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013;65(1):1–11. [2] Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF, et al. 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(1): 270–81.
Please cite this article as: Bouabdallaoui N, et al, Cardiogenic shock, asthma, and hypereosinophilia, Am J Emerg Med (2014), http://dx.doi.org/ 10.1016/j.ajem.2014.08.019