case report
Wien Klin Wochenschr DOI 10.1007/s00508-016-1051-4
Acute myocardial infarction as a manifestation of systemic vasculitis Martin Reindl · Sebastian Johannes Reinstadler · Hans-Josef Feistritzer · Agnes Mayr · Gert Klug · Peter Marschang · Bernhard Metzler
Received: 10 June 2016 / Accepted: 20 June 2016 © Springer-Verlag Wien 2016
Summary Acute myocardial infarction (AMI) is one of the major causes of mortality and morbidity worldwide. In Central Europe, causes of AMI other than atherosclerosis are unusual. Coronary artery vasculitis is one potential non-atherosclerotic process causing AMI. Herein, the authors depict a very rare case of AMI as a clinical manifestation of polyarteritis nodosa (PAN), a necrotizing systemic vasculitis. A 49year-old male patient presented to our clinic with abdominal pain and markedly elevated concentrations of C-reactive protein, creatinine and high-sensitivity cardiac troponin T. Electrocardiography showed new repolarization abnormalities in aVF and III. Besides PAN-typical angiographic findings, including bilateral renal artery microaneuryms as well as different arterial occlusions, coronary angiography displayed a complete thrombotic occlusion of the right coronary artery without any other coronary pathology. The present case report demonstrates AMI as very rare but deleterious complication in patients suffering from PAN, and highlights that this life-threatening event can occur even at a very early stage of PANrelated coronary affection. Keywords Polyarteritis nodosa · Myocardial infarction · Inflammation · Coronary angiography · Magnetic resonance imaging
M. Reindl · S. J. Reinstadler · H.-J. Feistritzer · G. Klug · P. Marschang · Univ.-Doz. Dr. B. Metzler () University Clinic of Internal Medicine III, Cardiology and Angiology, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria [email protected] A. Mayr University Clinic of Radiology, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria
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Introduction Despite substantial improvements in therapeutic strategies over the last decades, acute myocardial infarction (AMI) still remains one of the leading causes of death and morbidity worldwide. In more than 80 %, the pathogenesis of AMI is based on progressive atherosclerosis with subsequent plaque rupture [1]. One potential but relatively uncommon cause of AMI besides atherosclerosis is coronary artery vasculitis. Polyarteritis nodosa (PAN) is a necrotizing systemic vasculitis of small- to medium-sized vessels characterized by microaneurysms and arterial occlusions. Cardiac involvement of PAN was reported to occur in 4 to 30 % [2], mainly manifesting as congestive heart failure. However, AMI as a clinical manifestation of PAN is extremely rare.
Case report A 49-year-old man presented to our clinic in January 2016 due to abdominal pain for approximately 24 hours. Blood analyses showed markedly elevated levels of C-reactive protein (CRP; 196 mg/l), creatinine (1.22 mg/dl) and high-sensitivity cardiac troponin T (1541 ng/l). Electrocardiography displayed sinus rhythm with new repolarization abnormalities (inverted T-waves) in aVF and III; however, no typical cardiac symptoms such as angina pectoris were reported. Due to severe abdominal pain in combination with the laboratory findings, a computed tomography (CT) scan of the trunk was performed revealing multiple renal (Fig. 1a, stars) and splenic infarctions, as well as an occlusion of the right coronary artery (RCA). Due to the embolic-like pattern in the present case, an infective endocarditis was suspected. However, in repeated transoesophageal echocardiographies, no signs of endocarditis were detected and blood cultures
Acute myocardial infarction as a manifestation of systemic vasculitis
case report
Fig. 1 a Abdominal CT scan showing multiple infarctions of the right kidney (stars). b MRI scan of the head exhibiting supratentorial cerebral infarctions (stars). c Magnetic resonance angiography with bilateral renal artery microaneurysms (arrows). d Magnetic resonance angiography showing bilateral occlusion of the popliteal arteries (arrows). e, f Coronary angiography revealing a thrombotic occlusion of the right coronary artery (arrow) without any atherosclerotic finding in the other coronary arteries
Fig. 2 Late gadolinium enhancement cardiac magnetic resonance displaying a myocardial infarction of the inferior wall (arrow)
remained negative. Further diagnostics revealed several cerebral infarctions (Fig. 1b, stars), bilateral renal artery microaneurysms (Fig. 1c, arrows) and bilateral popliteal artery occlusions (Fig. 1d, arrows). These findings indicated the strong suspicion of a present systemic vasculitis, in particular medium vessel vasculitis. Indeed, the presence of four positive American College of Rheumatology (ACR) criteria lead to the diagnosis of PAN: (1) typical angiographic abnormalities (occlusions, microaneurysms), (2) elevated creatinine concentration, (3) diastolic blood pressure >90 mmHG and (4) weight loss ≥4 kg (three positive ACR criteria are required for diagnosis) [3]. After initiation of immunosuppressive treatment (methylprednisolone) and decrease of CRP levels, coronary angiography (CAG) was performed. Notably, CAG confirmed a complete thrombotic occlusion of the RCA (Fig. 1e, arrow) without any atherosclerotic lesion in the left anterior descending or circumflex artery (Fig. 1f). This angiographic finding of a nonatherosclerotic coronary pattern was consistent with the presence of vasculitis as underlying cause of the occlusion. In line with the CAG results, cardiac magnetic resonance imaging showed a partially transmural myocardial infarction of the inferior wall (Fig. 2, arrow). After stent implantation and continuation
Acute myocardial infarction as a manifestation of systemic vasculitis
K
case report
of immunosuppressive medication, the patient was discharged in an improved clinical condition a few weeks after CAG.
Discussion The present clinical report depicts a very rare case of PAN manifesting as AMI with a total thrombotic occlusion of the RCA without any other coronary artery pathology. In European countries, PAN is a very rare disease showing an annual incidence of 0–1.6 cases/ million inhabitants [2]. This specific form of systemic vasculitis shows necrotizing inflammatory lesions of small and medium-sized vessels, resulting in microaneurysm formation, rupture and occlusion of inflamed arteries. These inflammatory processes can eventually lead to tissue ischemia or haemorrhage in multiple organs, with a variety of clinical presentations. The skin and the peripheral nervous system are the most frequently affected systems in PAN [2]. Furthermore, renal manifestations were stated as relatively common, typically presenting with hypertension and renal insufficiency [4]. Cardiac involvement in patients suffering from PAN was demonstrated previously and particularly congestive heart failure was reported as a relatively frequent complication [5]. The pathogenesis of heart failure in PAN patients is multifactorial and not yet entirely clarified. Active or healed vasculitis of the coronary arteries as well as renovascular hypertension were identified as major determinants [6]. Nevertheless, the occurrence of manifest AMI is extremely rare in PAN patients [7]. In previous reports, PAN-related AMI was found particularly in patients displaying severe coronary artery affections including multiple aneurysms [8, 9]. In contrast, the CAG of our patient disclosed, besides the thrombotic occlusion of the RCA, mostly normal
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coronary arteries without any detectable aneurysm or other pathology. These findings indicate a very early stage of PAN-related coronary affection. Accordingly, the present case not only demonstrates AMI as a very rare complication of PAN, but also reveals that this complication might occur at a very early phase of coronary artery involvement. Conflict of interest M. Reindl, S.J. Reinstadler, H.-J. Feistritzer, P. Marschang, B. Metzler, A. Mayr and G. Klug declare that they have no competing interests.
References 1. Burke AP, Virmani R. Pathophysiology of acute myocardial infarction. Med Clin North Am. 2007;91:553–72. 2. Hernandez-Rodriguez J, Alba MA, Prieto-Gonzalez S, et al. Diagnosis and classification of polyarteritis nodosa. J Autoimmun. 2014;48–49:84–9. 3. Lightfoot RW Jr., Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33:1088–93. 4. Howard T, Ahmad K, Swanson JA, et al. Polyarteritis nodosa. Tech Vasc Interv Radiol. 2014;17:247–51. 5. Chung DC, Choi JE, Song YK, et al. Polyarteritis nodosa complicated by chronic total occlusion accompanying aneurysms on all coronary arteries. Korean Circ J. 2012;42:568–70. 6. Rajani RM, Dalvi BV, D’Silva SA, et al. Acute myocardial infarction with normal coronary arteries in a case of polyarteritis nodosa: possible role of coronary artery spasm. Postgrad Med J. 1991;67:78–80. 7. McWilliams ET, Khonizy W, Jameel A. Polyarteritis nodosa presenting as acute myocardial infarction in a young man: importance of invasive angiography. Heart. 2013;99:1219. 8. Yuce M, Davutoglu V, Sari I, et al. Polyarteritis nodosa with multiple coronary aneurysms presenting as acute myocardial infarction. Am J Med Sci. 2011;341:409. 9. Kastner D, Gaffney M, Tak T. Polyarteritis nodosa and myocardial infarction. Can J Cardiol. 2000;16:515–8.
Acute myocardial infarction as a manifestation of systemic vasculitis
Wien Klin Wochenschr DOI 10.1007/s00508-016-1051-4
Acute myocardial infarction as a manifestation of systemic vasculitis Martin Reindl · Sebastian Johannes Reinstadler · Hans-Josef Feistritzer · Agnes Mayr · Gert Klug · Peter Marschang · Bernhard Metzler
Received: 10 June 2016 / Accepted: 20 June 2016 © Springer-Verlag Wien 2016
Summary Acute myocardial infarction (AMI) is one of the major causes of mortality and morbidity worldwide. In Central Europe, causes of AMI other than atherosclerosis are unusual. Coronary artery vasculitis is one potential non-atherosclerotic process causing AMI. Herein, the authors depict a very rare case of AMI as a clinical manifestation of polyarteritis nodosa (PAN), a necrotizing systemic vasculitis. A 49year-old male patient presented to our clinic with abdominal pain and markedly elevated concentrations of C-reactive protein, creatinine and high-sensitivity cardiac troponin T. Electrocardiography showed new repolarization abnormalities in aVF and III. Besides PAN-typical angiographic findings, including bilateral renal artery microaneuryms as well as different arterial occlusions, coronary angiography displayed a complete thrombotic occlusion of the right coronary artery without any other coronary pathology. The present case report demonstrates AMI as very rare but deleterious complication in patients suffering from PAN, and highlights that this life-threatening event can occur even at a very early stage of PANrelated coronary affection. Keywords Polyarteritis nodosa · Myocardial infarction · Inflammation · Coronary angiography · Magnetic resonance imaging
M. Reindl · S. J. Reinstadler · H.-J. Feistritzer · G. Klug · P. Marschang · Univ.-Doz. Dr. B. Metzler () University Clinic of Internal Medicine III, Cardiology and Angiology, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria [email protected] A. Mayr University Clinic of Radiology, Medical University of Innsbruck, Anichstraße 35, 6020 Innsbruck, Austria
K
Introduction Despite substantial improvements in therapeutic strategies over the last decades, acute myocardial infarction (AMI) still remains one of the leading causes of death and morbidity worldwide. In more than 80 %, the pathogenesis of AMI is based on progressive atherosclerosis with subsequent plaque rupture [1]. One potential but relatively uncommon cause of AMI besides atherosclerosis is coronary artery vasculitis. Polyarteritis nodosa (PAN) is a necrotizing systemic vasculitis of small- to medium-sized vessels characterized by microaneurysms and arterial occlusions. Cardiac involvement of PAN was reported to occur in 4 to 30 % [2], mainly manifesting as congestive heart failure. However, AMI as a clinical manifestation of PAN is extremely rare.
Case report A 49-year-old man presented to our clinic in January 2016 due to abdominal pain for approximately 24 hours. Blood analyses showed markedly elevated levels of C-reactive protein (CRP; 196 mg/l), creatinine (1.22 mg/dl) and high-sensitivity cardiac troponin T (1541 ng/l). Electrocardiography displayed sinus rhythm with new repolarization abnormalities (inverted T-waves) in aVF and III; however, no typical cardiac symptoms such as angina pectoris were reported. Due to severe abdominal pain in combination with the laboratory findings, a computed tomography (CT) scan of the trunk was performed revealing multiple renal (Fig. 1a, stars) and splenic infarctions, as well as an occlusion of the right coronary artery (RCA). Due to the embolic-like pattern in the present case, an infective endocarditis was suspected. However, in repeated transoesophageal echocardiographies, no signs of endocarditis were detected and blood cultures
Acute myocardial infarction as a manifestation of systemic vasculitis
case report
Fig. 1 a Abdominal CT scan showing multiple infarctions of the right kidney (stars). b MRI scan of the head exhibiting supratentorial cerebral infarctions (stars). c Magnetic resonance angiography with bilateral renal artery microaneurysms (arrows). d Magnetic resonance angiography showing bilateral occlusion of the popliteal arteries (arrows). e, f Coronary angiography revealing a thrombotic occlusion of the right coronary artery (arrow) without any atherosclerotic finding in the other coronary arteries
Fig. 2 Late gadolinium enhancement cardiac magnetic resonance displaying a myocardial infarction of the inferior wall (arrow)
remained negative. Further diagnostics revealed several cerebral infarctions (Fig. 1b, stars), bilateral renal artery microaneurysms (Fig. 1c, arrows) and bilateral popliteal artery occlusions (Fig. 1d, arrows). These findings indicated the strong suspicion of a present systemic vasculitis, in particular medium vessel vasculitis. Indeed, the presence of four positive American College of Rheumatology (ACR) criteria lead to the diagnosis of PAN: (1) typical angiographic abnormalities (occlusions, microaneurysms), (2) elevated creatinine concentration, (3) diastolic blood pressure >90 mmHG and (4) weight loss ≥4 kg (three positive ACR criteria are required for diagnosis) [3]. After initiation of immunosuppressive treatment (methylprednisolone) and decrease of CRP levels, coronary angiography (CAG) was performed. Notably, CAG confirmed a complete thrombotic occlusion of the RCA (Fig. 1e, arrow) without any atherosclerotic lesion in the left anterior descending or circumflex artery (Fig. 1f). This angiographic finding of a nonatherosclerotic coronary pattern was consistent with the presence of vasculitis as underlying cause of the occlusion. In line with the CAG results, cardiac magnetic resonance imaging showed a partially transmural myocardial infarction of the inferior wall (Fig. 2, arrow). After stent implantation and continuation
Acute myocardial infarction as a manifestation of systemic vasculitis
K
case report
of immunosuppressive medication, the patient was discharged in an improved clinical condition a few weeks after CAG.
Discussion The present clinical report depicts a very rare case of PAN manifesting as AMI with a total thrombotic occlusion of the RCA without any other coronary artery pathology. In European countries, PAN is a very rare disease showing an annual incidence of 0–1.6 cases/ million inhabitants [2]. This specific form of systemic vasculitis shows necrotizing inflammatory lesions of small and medium-sized vessels, resulting in microaneurysm formation, rupture and occlusion of inflamed arteries. These inflammatory processes can eventually lead to tissue ischemia or haemorrhage in multiple organs, with a variety of clinical presentations. The skin and the peripheral nervous system are the most frequently affected systems in PAN [2]. Furthermore, renal manifestations were stated as relatively common, typically presenting with hypertension and renal insufficiency [4]. Cardiac involvement in patients suffering from PAN was demonstrated previously and particularly congestive heart failure was reported as a relatively frequent complication [5]. The pathogenesis of heart failure in PAN patients is multifactorial and not yet entirely clarified. Active or healed vasculitis of the coronary arteries as well as renovascular hypertension were identified as major determinants [6]. Nevertheless, the occurrence of manifest AMI is extremely rare in PAN patients [7]. In previous reports, PAN-related AMI was found particularly in patients displaying severe coronary artery affections including multiple aneurysms [8, 9]. In contrast, the CAG of our patient disclosed, besides the thrombotic occlusion of the RCA, mostly normal
K
coronary arteries without any detectable aneurysm or other pathology. These findings indicate a very early stage of PAN-related coronary affection. Accordingly, the present case not only demonstrates AMI as a very rare complication of PAN, but also reveals that this complication might occur at a very early phase of coronary artery involvement. Conflict of interest M. Reindl, S.J. Reinstadler, H.-J. Feistritzer, P. Marschang, B. Metzler, A. Mayr and G. Klug declare that they have no competing interests.
References 1. Burke AP, Virmani R. Pathophysiology of acute myocardial infarction. Med Clin North Am. 2007;91:553–72. 2. Hernandez-Rodriguez J, Alba MA, Prieto-Gonzalez S, et al. Diagnosis and classification of polyarteritis nodosa. J Autoimmun. 2014;48–49:84–9. 3. Lightfoot RW Jr., Michel BA, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990;33:1088–93. 4. Howard T, Ahmad K, Swanson JA, et al. Polyarteritis nodosa. Tech Vasc Interv Radiol. 2014;17:247–51. 5. Chung DC, Choi JE, Song YK, et al. Polyarteritis nodosa complicated by chronic total occlusion accompanying aneurysms on all coronary arteries. Korean Circ J. 2012;42:568–70. 6. Rajani RM, Dalvi BV, D’Silva SA, et al. Acute myocardial infarction with normal coronary arteries in a case of polyarteritis nodosa: possible role of coronary artery spasm. Postgrad Med J. 1991;67:78–80. 7. McWilliams ET, Khonizy W, Jameel A. Polyarteritis nodosa presenting as acute myocardial infarction in a young man: importance of invasive angiography. Heart. 2013;99:1219. 8. Yuce M, Davutoglu V, Sari I, et al. Polyarteritis nodosa with multiple coronary aneurysms presenting as acute myocardial infarction. Am J Med Sci. 2011;341:409. 9. Kastner D, Gaffney M, Tak T. Polyarteritis nodosa and myocardial infarction. Can J Cardiol. 2000;16:515–8.
Acute myocardial infarction as a manifestation of systemic vasculitis
