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Churg-Strauss syndrome masquerading as an acute coronary syndrome
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
Churg-Strauss syndrome masquerading as an acute coronary syndrome: a case report☆ Abstract Churg-Strauss Syndrome (CSS) is a rare vasculitis with multiorgan involvement. Cardiac manifestations are common causing serious complications. We report a case of CSS masquerading as a non-ST elevation myocardial infarction with heart failure. CSS should be considered in the differential diagnosis of an acute coronary syndrome (ACS) with normal coronary arteries when history of asthma, peripheral eosinophilia and multisystemic involvement is present.
A 62-year-old woman was referred to the emergency department from a remote health care facility with the diagnosis of a non–STelevation myocardial infarction. Chest pain was severe and lasted for 20 minutes. She also reported dyspnea of recent onset in moderate exertion and a drop of the right foot since last month. History included asthma under intermittent steroid treatment while she reported no cardiac disease. She was referred at our hospital 48 hours after symptom onset. At presentation she was mildly dyspnoic, afebrile, with a blood pressure of 120/80 mmHg and a regular heart rate of 100 beat/min. Examination revealed S3, systolic murmur in the apex of 2/6, mild crackles at lung bases and bilateral ankle edema. Resting 12-lead electrocardiogram showed diffuse repolarisation abnormalities and poor R wave progression in precordial leads V1 to V3 (Fig. 1). Blood testing revealed elevated troponin levels (0.42 ng/mL; normal value b0.07 ng/mL) and a marked increase of eosinophils in leukocyte count (25%). Echocardiography reported moderately impaired left ventricular ejection fraction (35%) and akinesis of the apex and the mid-apical interventricular septum. Thickening and impaired mobility of the posterior mitral leaflet were also noted resulting in severe mitral regurgitation (MR) (Fig. 2A). Echocardiography performed a year before was normal. She was started on aspirin, clopidogrel, enoxaparin, betablocker, angiotensin-enzyme inhibitor, statin, spironolactone, and furosemide and a coronary angiogram was scheduled. Twenty-four hours after admission, the patient developed dysarthria and right hemiparesis. Brain computed tomographic (CT) scan revealed ischemic stroke. Patient's level of consciousness and dyspnea worsened leading to intubation. Chest CT scan detected mild pleural effusion, bilateral ground glass opacities and sub-pleural airspace consolidation suggestive of pulmonary infiltrates (Fig. 2B). Neurology and rheumatology consultation were obtained. CSS was considered likely and prednisone with cyclophosphamide were initiated. Patient improved ☆ Disclosures: The authors have no conflicts to disclose. Patient consent was obtained.
and was extubated after a week. Eosinophils count normalized within few days. Following the completion of four cycles, cyclophoshamide was stopped, and azathioprine was added to prednisone. Coronary angiography revealed normal coronary arteries. Clopidogrel, enoxaparine and statin were discontinued. Reevaluation echocardiography showed no appreciable changes and patient was discharged in good condition. During follow-up, she was in full remission. However, due to severe MR and dyspnea (New York Heart Association class II) she underwent mitral valve replacement with a mechanical prosthesis resulting in postoperative improvement. CSS is a multisystem vasculitis of the small and medium sized arteries characterized by chronic rhinosinusitis, asthma and peripheral blood eosinophilia. Commonly lung and skin are involved, however, cardiovascular, gastrointestinal, renal and nervous system can be affected. CSS has three stages: prodromal of asthma and rhinosinusitis, eosinophilic, and vasculitic phase [1,2]. The American College of Rheumatology proposed the following diagnostic criteria (established when four out of six are met): asthma, eosinophils on the leukocyte count greater than 10%, mononeuropathy or polyneuropathy, migratory or transient pulmonary opacities detected radiographically, paranasal sinus abnormality and evidence of eosinophilic infiltration in extravascular organ [3]. Our patient fulfilled the first four of the aforementioned criteria meeting the diagnosis of CSS. Antineutrophil cytoplasmic antibodies may play a role in CSS although positivity occurs in 40-60% [4]. Differential diagnosis includes hypereosinophilic syndrome where asthma presence is unlikely [2]. Cardiac involvement is common causing serious complications, accounting for 50% of deaths. Manifestations include heart failure, valvulopathy, pericarditis, arrhythmias, and coronary vasculitis. They are more frequent in negative–antineutrophil cytoplasmic antibody CSS [2]. CSS mimicking an ACS is rare. Systemic inflammation may cause coronary vasculitis and atheromatosis. Our patient, had angiographically normal coronaries. We assumed that coronary vasculitis is the cause of the ACS. Moreover, impaired left ventricular ejection fraction and severe MR underscore an additional CSS cardiac manifestation. Myocardium eosinophilic infiltration causes inflammation, fibrosis, and impaired cardiac function. Early cardiac involvement may be subclinical. Echocardiography and cardiac magnetic resonance imaging are helpful in the diagnosis of cardiac involvement. Early treatment may have protective cardiovascular effects [2,5]. Therapy includes immunosuppressive agents. In our patient, aspirin and standard regimen for heart failure were added. Finally, she
0735-6757/© 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Triantafyllis AS, et al, Churg-Strauss syndrome masquerading as an acute coronary syndrome: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.08.003
2
A.S. Triantafyllis et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 1. Admission resting 12-lead electrocardiogram showing diffuse repolarisation abnormalities and poor R wave progression in precordial leads V1 to V3.
Fig. 2. A, Color flow Doppler showing severe mitral regurgitation. B, Chest CT-scan revealing bilateral ground glass opacities.
underwent mitral valve replacement with a mechanical prosthesis resulting in postoperative improvement. CSS should be considered in the differential diagnosis of ACS with normal coronary arteries when history of asthma, peripheral eosinophilia and multiorgan involvement is present. Andreas S. Triantafyllis MD, PhD 1,⁎ Eleftherios A. Sakadakis MD, PhD 1 Argyro Papafilippaki MD University of Athens, Second Cardiology Department Attikon University Hospital, Athens, Greece ⁎ Corresponding author. Second Cardiology Department Attikon University Hospital Rimini 1, Haidari 12462 Athens Greece. Tel.: +30 2105832355; fax: +30 2105832351. E-mail addresses: [email protected] [email protected] 1
Joint first authors
Pelagia Katsimbri MD University of Athens Fourth Internal Medicine Department Attikon University Hospital Athens, Greece
Fotios Panou MD, PhD Maria Anastasiou-Nana MD, PhD Ioannis Lekakis MD, PhD University of Athens, Second Cardiology Department Attikon University Hospital, Athens, Greece
http://dx.doi.org/10.1016/j.ajem.2014.08.003
References [1] Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009;23 (3):355–66. [2] Ungprasert P, Srivali N, Cheungpasitporn W, Schaeffer CS. Is it acute coronary syndrome or Churg-Strauss syndrome? Am J Emerg Med 2013;31(1):270.e5–8. [3] Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome. Arthritis Rheum 1990;33(8):1094–100. [4] 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;52:2926–35. [5] 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.
Please cite this article as: Triantafyllis AS, et al, Churg-Strauss syndrome masquerading as an acute coronary syndrome: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.08.003
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Churg-Strauss syndrome masquerading as an acute coronary syndrome: a case report☆ Abstract Churg-Strauss Syndrome (CSS) is a rare vasculitis with multiorgan involvement. Cardiac manifestations are common causing serious complications. We report a case of CSS masquerading as a non-ST elevation myocardial infarction with heart failure. CSS should be considered in the differential diagnosis of an acute coronary syndrome (ACS) with normal coronary arteries when history of asthma, peripheral eosinophilia and multisystemic involvement is present.
A 62-year-old woman was referred to the emergency department from a remote health care facility with the diagnosis of a non–STelevation myocardial infarction. Chest pain was severe and lasted for 20 minutes. She also reported dyspnea of recent onset in moderate exertion and a drop of the right foot since last month. History included asthma under intermittent steroid treatment while she reported no cardiac disease. She was referred at our hospital 48 hours after symptom onset. At presentation she was mildly dyspnoic, afebrile, with a blood pressure of 120/80 mmHg and a regular heart rate of 100 beat/min. Examination revealed S3, systolic murmur in the apex of 2/6, mild crackles at lung bases and bilateral ankle edema. Resting 12-lead electrocardiogram showed diffuse repolarisation abnormalities and poor R wave progression in precordial leads V1 to V3 (Fig. 1). Blood testing revealed elevated troponin levels (0.42 ng/mL; normal value b0.07 ng/mL) and a marked increase of eosinophils in leukocyte count (25%). Echocardiography reported moderately impaired left ventricular ejection fraction (35%) and akinesis of the apex and the mid-apical interventricular septum. Thickening and impaired mobility of the posterior mitral leaflet were also noted resulting in severe mitral regurgitation (MR) (Fig. 2A). Echocardiography performed a year before was normal. She was started on aspirin, clopidogrel, enoxaparin, betablocker, angiotensin-enzyme inhibitor, statin, spironolactone, and furosemide and a coronary angiogram was scheduled. Twenty-four hours after admission, the patient developed dysarthria and right hemiparesis. Brain computed tomographic (CT) scan revealed ischemic stroke. Patient's level of consciousness and dyspnea worsened leading to intubation. Chest CT scan detected mild pleural effusion, bilateral ground glass opacities and sub-pleural airspace consolidation suggestive of pulmonary infiltrates (Fig. 2B). Neurology and rheumatology consultation were obtained. CSS was considered likely and prednisone with cyclophosphamide were initiated. Patient improved ☆ Disclosures: The authors have no conflicts to disclose. Patient consent was obtained.
and was extubated after a week. Eosinophils count normalized within few days. Following the completion of four cycles, cyclophoshamide was stopped, and azathioprine was added to prednisone. Coronary angiography revealed normal coronary arteries. Clopidogrel, enoxaparine and statin were discontinued. Reevaluation echocardiography showed no appreciable changes and patient was discharged in good condition. During follow-up, she was in full remission. However, due to severe MR and dyspnea (New York Heart Association class II) she underwent mitral valve replacement with a mechanical prosthesis resulting in postoperative improvement. CSS is a multisystem vasculitis of the small and medium sized arteries characterized by chronic rhinosinusitis, asthma and peripheral blood eosinophilia. Commonly lung and skin are involved, however, cardiovascular, gastrointestinal, renal and nervous system can be affected. CSS has three stages: prodromal of asthma and rhinosinusitis, eosinophilic, and vasculitic phase [1,2]. The American College of Rheumatology proposed the following diagnostic criteria (established when four out of six are met): asthma, eosinophils on the leukocyte count greater than 10%, mononeuropathy or polyneuropathy, migratory or transient pulmonary opacities detected radiographically, paranasal sinus abnormality and evidence of eosinophilic infiltration in extravascular organ [3]. Our patient fulfilled the first four of the aforementioned criteria meeting the diagnosis of CSS. Antineutrophil cytoplasmic antibodies may play a role in CSS although positivity occurs in 40-60% [4]. Differential diagnosis includes hypereosinophilic syndrome where asthma presence is unlikely [2]. Cardiac involvement is common causing serious complications, accounting for 50% of deaths. Manifestations include heart failure, valvulopathy, pericarditis, arrhythmias, and coronary vasculitis. They are more frequent in negative–antineutrophil cytoplasmic antibody CSS [2]. CSS mimicking an ACS is rare. Systemic inflammation may cause coronary vasculitis and atheromatosis. Our patient, had angiographically normal coronaries. We assumed that coronary vasculitis is the cause of the ACS. Moreover, impaired left ventricular ejection fraction and severe MR underscore an additional CSS cardiac manifestation. Myocardium eosinophilic infiltration causes inflammation, fibrosis, and impaired cardiac function. Early cardiac involvement may be subclinical. Echocardiography and cardiac magnetic resonance imaging are helpful in the diagnosis of cardiac involvement. Early treatment may have protective cardiovascular effects [2,5]. Therapy includes immunosuppressive agents. In our patient, aspirin and standard regimen for heart failure were added. Finally, she
0735-6757/© 2014 Elsevier Inc. All rights reserved.
Please cite this article as: Triantafyllis AS, et al, Churg-Strauss syndrome masquerading as an acute coronary syndrome: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.08.003
2
A.S. Triantafyllis et al. / American Journal of Emergency Medicine xxx (2014) xxx–xxx
Fig. 1. Admission resting 12-lead electrocardiogram showing diffuse repolarisation abnormalities and poor R wave progression in precordial leads V1 to V3.
Fig. 2. A, Color flow Doppler showing severe mitral regurgitation. B, Chest CT-scan revealing bilateral ground glass opacities.
underwent mitral valve replacement with a mechanical prosthesis resulting in postoperative improvement. CSS should be considered in the differential diagnosis of ACS with normal coronary arteries when history of asthma, peripheral eosinophilia and multiorgan involvement is present. Andreas S. Triantafyllis MD, PhD 1,⁎ Eleftherios A. Sakadakis MD, PhD 1 Argyro Papafilippaki MD University of Athens, Second Cardiology Department Attikon University Hospital, Athens, Greece ⁎ Corresponding author. Second Cardiology Department Attikon University Hospital Rimini 1, Haidari 12462 Athens Greece. Tel.: +30 2105832355; fax: +30 2105832351. E-mail addresses: [email protected] [email protected] 1
Joint first authors
Pelagia Katsimbri MD University of Athens Fourth Internal Medicine Department Attikon University Hospital Athens, Greece
Fotios Panou MD, PhD Maria Anastasiou-Nana MD, PhD Ioannis Lekakis MD, PhD University of Athens, Second Cardiology Department Attikon University Hospital, Athens, Greece
http://dx.doi.org/10.1016/j.ajem.2014.08.003
References [1] Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009;23 (3):355–66. [2] Ungprasert P, Srivali N, Cheungpasitporn W, Schaeffer CS. Is it acute coronary syndrome or Churg-Strauss syndrome? Am J Emerg Med 2013;31(1):270.e5–8. [3] Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome. Arthritis Rheum 1990;33(8):1094–100. [4] 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;52:2926–35. [5] 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.
Please cite this article as: Triantafyllis AS, et al, Churg-Strauss syndrome masquerading as an acute coronary syndrome: a case report, Am J Emerg Med (2014), http://dx.doi.org/10.1016/j.ajem.2014.08.003