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Hypereosinophilic syndrome

The hypereosinophilic syndrome is a disease process characterized by a persistently elevated eosinophil count (≥ 1500 eosinophils/mm3) in the blood for at least six months without any recognizable cause after a careful workup, with evidence of involvement of either the heart, nervous system, or bone marrow. more...

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There are two forms of the hypereosinophilic syndrome: Endomyocardial fibrosis and Loeffler's endocarditis. Endomyocardial fibrosis (also known as Davies disease) is seen in Africa and South America, while Loeffler's endocarditis does not have any geographic predisposition.

In both forms of the hypereosinophilic syndrome, the eosinophilia causes infiltration of the myocardium of the heart, which leads to fibrotic thickening of portions of the heart. The portions of the heart most effected by this disease are the apex of the left and right ventricles, fibrotic infiltrations may involve the mitral or tricuspid valves. Because of the infiltrative nature of the disease process, the cavity of the ventricles of the heart diminish in size, causing an obliterative cardiomyopathy and restriction to the inflow of blood in to the chambers of the heart. Ventricular mural thromb may develop.

Chronic eosinophilic leukemia (CEL) is a myeloproliferative disease which shares many common characteristics with hypereosinophilic syndrome. Many cases of CEL have a characteristic gene rearrangement , FIP1L1/PDGFRA, caused by a sub-micoscopic deletion of ~800 thousand base pairs of DNA on chromosome 4. The FIP1L1/PDGFRA fusion gene causes consitutive activation of the platelet derived growth factor receptor - alpha (PDGFRA). FIP1L1/PDGFRA-positive patients respond well to treatment with the tyrosine kinase inhibitor drug, imatinib mesylate (Gleevec® or Glivec®).

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A case of sudden cardiac death due to isolated eosinophilic coronary arteritis
From CHEST, 8/1/05 by Philipp M. Lepper

Spontaneous coronary artery dissection is a very rare event and occurs most often in young women following childbirth. It is also known as a rare focal complication in Churg-Strauss syndrome. Here, we present the case of a 43-year-old woman who died after spontaneous dissection of all three coronary arteries. The microscopic examination of coronary vessels showed severe eosinophilic infiltrations, whereas all extracardiac (medium-vessel and large-vessel) arteries were intact and free of inflammatory cells. Her history did not reveal allergy, asthma, or eosinophilia. To the best of our knowledge, this is the first case of spontaneous coronary dissection involving all coronary arteries without a history of Churg-Strauss syndrome or hypereosinophilic syndrome. (CHEST 2005; 128:1047-1050)

Key words: arteritis; Churg-Strauss syndrome; sudden death

Abbreviations: LAD = left anterior descending; LV = left ventricle, ventricular; PAN = panarteritis nodosa

**********

Inflammation of the vessel wall occurs in a number of disorders, including Kawasaki disease, Churg-Strauss syndrome, Behcet syndrome, rheumatoid arthritis, syphilis, tuberculosis, and autoimmunity to heat-shock protein 65. (1-6) Dissections of coronary arteries are increasingly recognized as a cause of sudden cardiac death in apparently healthy individuals. Women are affected more frequently (85% of cases reported), and approximately 35% of coronary artery dissections occur < 3 months postpartum. Dissections most often occur in the left coronary artery system (approximately 75%), primarily the left main artery or left anterior descending (LAD) artery (approximately 80%). (7,8) Eosinophilic infiltration of the coronary arteries has been described (9) as a limited form of Churg-Strauss syndrome in patients with a lifelong history of allergies to (often) numerous agents. (10) Panarteritis nodosa (PAN), a medium-sized-vessel vasculitis, has been described as a cause of myocardial infarction and angina. (3) However, PAN also involves, at the same time, arteries other than the coronary arteries. In most of the arterial inflammatory disorders, cystic necroses in the vessel wall can be found. (11) There have been numerous case reports (12-16) on inflammatory alterations in coronary arteries as a cause of sudden cardiac death. Rarely, the affected coronary arteries develop aneurysms. (17,18) Previously, cases of strictly isolated eosinophilic coronary arteritis have been reported (9,10,19); however, these occurrences were preceded by signs or symptoms of allergy, asthma, and dyspnea. The causes of inflammatory diseases of the coronary arteries, as reported in the literature, are mainly PAN, (3) Takayasu arteritis, (20) and rheumatic disease. (6) Giant cell arteritis affects persons > 50 years of age almost exclusively, and the disease risk is highest among those who are > 75 years of age. (21) Systemic inflammation is present in almost all patients with giant cell arteritis. (22) Here, we report a case in which the patient had none of the alterations described as predisposing a person to spontaneous coronary dissection.

CASE REPORT

A 43-year-old woman died of multiple spontaneous coronary artery dissections caused by eosinophilic arteritis in all three major coronary arteries. Six years prior to death, she had presented with stress-induced dyspnea and a history of hypertension, and was referred to a cardiologist. There was no history of asthma or a positive family history of coronary artery disease. A physical examination was without pathologic findings, and especially, no heart murmur was present (cardiovascular risk factors were obesity and hypertension, but there was no history of diabetes and the total cholesterol/high-density lipoprotein ratio was normal). No enzyme defects related to collagen metabolism were noted. Echocardiography revealed a dilated left ventricle (LV) [ie, end-diastolic diameter, 67 mm] with slightly impaired LV function and mild mitral valve insufficiency. ECG showed sinus rhythm and left-axis deviation at a resting heart rate of 61 beats/min. No bundle branch block was seen. An exercise stress test was performed to a 100-W workload with an adequate heart rate and a hypertensive BP response, and no signs and symptoms of angina, ST-segment depression, or rhythm disorders.

One year later, the exercise stress test was repeated, and the patient was again asymptomatic at 75 W. Because of atypical chest pain, a heart catheterization was performed, which revealed normal hemodynamics. All coronary arteries were free from visible atherosclerotic lesions. The patient received therapy with a combination of beta-blocker, diuretic, and angiotensin-II receptor blocker. Furthermore, she started to receive therapy with aspirin (100 mg/d).

The patient had planned to undergo an abdominal hysterectomy because of uterus myomatosus with polymennorrhea; therefore, aspirin therapy was discontinued. Five days before hospital admission, the patient's hemoglobin level was 13.6 g/dL but was found to be 8.6 g/dL when the patient was hospitalized. C-reactive protein level and erythrocyte sedimentation rate were normal on hospital admission. The patient underwent two blood transfusions and was operated on successfully the following morning. The day after surgery, she complained about chest pain (right sided pressure, not typical for angina). An ECG was performed, which showed no changes compared with previous ECGs. Blood tests for myocardial necrosis markers (ie, creatinine kinase, creatinine kinase-MB, and troponin I) had negative results. No specific therapy was initiated at that time. Regular postoperative care was continued. Two days later, the patient was found dead in her bed at 4:00 AM. An autopsy revealed a regular status after the hysterectomy without signs of infection or bleeding. The abdominal organs were without pathologic findings, especially without signs of necrotizing vasculitis, granulomas with eosinophilic necrosis, or tissue infiltration by eosinophils. There was acute pulmonary congestion. The heart showed LV dilatation and compensatory hypertrophy, with a heart weight of 470 g. No valve diseases or anomalies of the coronary arteries were seen. An examination of the external surface of the heart revealed perivascular hemorrhage overlying all three epicordial arteries (Fig 1). There was no evidence of atherosclerosis either in the coronary vascular bed, or in the aorta or large aortic branches. Coronary artery dissection was confirmed histologically in serial transversal sections of all three vessels with a similar histology. The lumina were occluded due to proximal dissection between the media and adventitial layer with large intramural hematomas (Fig 2). The adventitial tissue was found to be infiltrated with inflammatory cells, predominantly eosinophils (Fig 3, 4). There was no fibrinoid necrosis of arterial walls, and no microorganisms were identified. There was also no evidence of cystic medianecrosis of the aorta or of the coronary arteries, and no visceral eosinophilia, extracardiac necrotizing arteritis, or granulomatosis were evident. In addition, the LAD artery revealed ferrous deposits under Perls staining (Prussian Blue stain), indicating a previous rupture of this branch leading to stenosis. The combination of LAD artery narrowing together with LV hypertrophy had caused ischemic cardiomyocyte damage, which was evidenced by the presence of abnormal CD56 positivity (23) and diffuse irregular interstitial myocardial fibrosis.

[FIGURES 1-4 OMITTED]

DISCUSSION

The ease of fatal isolated eosinophilic coronaritis presented here is of interest for several reasons. First, our histopathologic findings confirm the findings of a previous report, (8) which suggested that changes, mainly in the media of the coronary arteries, predisposes the patient to dissection, and that these changes are likely to be associated with eosinophilic infiltration. Eosinophils are capable of releasing cytotoxic substances and matrix metalloproteinases into the adjacent adventitia and media of the arteries, causing substantial weakening and focal necrosis (not present in this case). Nonlaminar shear forces may cause subsequent intimal disruption and mural dissection. In most eases, the etiology of an eosinophilic infiltrate remains elusive; however, it seems to be part of a systemic process. Contrary to previously described cases, (24,25) no systemic involvement was seen in the case presented here. There have been reports of spontaneous carotid artery dissections, but in these cases no such histopathologic findings have been reported. (26) Second, the case demonstrates the occurrence of noncontiguous dissections in all three main coronary arteries, suggesting a profound underlying change in vascular biology only in the affected vascular beds. The reasons for such a change are poorly understood in patients with conditions other than atherosclerosis, which to date has been considered to be a local inflammatory disorder of the vessel wall accompanied by a systemic inflammatory response. The fact that spontaneous coronary artery dissection is linked to the postpartum period suggests the involvement of endocrine factors. It has been postulated that hormonal changes associated with pregnancy weaken the coronary arterial wall. Subsequently, the strain of delivery may initiate fissuring in the intima, which proceeds slowly to dissection over a few days or weeks, and eventually coronary flow becomes compromised and fatal arrhythmia or myocardial infarction occurs. (7) In the present case, the woman was not postpartum; however, she had delivered four children (ages, 4 to 11 years), and, as we could show by Prussian blue staining, coronary arteries might have been affected through recurrent inflammation of the vessel wall and were prone to rupture. In summary, the etiology and precise mechanisms involved in the pathogenesis of eosinophilic arteritis of the coronary arteries is unknown but, in this ease, does not appear to be a part of a systemic vascular process. Hypersensitivity or an elusive autoimmune mechanism may be causative.

REFERENCES

(1) George J, Greenberg S, Barshack I, et al. Immunity to heat shock protein 65: an additional determinant in intimal thickening. Atherosclerosis 2003; 168:33-38

(2) George J, Shoenfeld Y, Gilburd B, et al. Requisite role for interleukin-4 in the acceleration of fatty streaks induced by heat shock protein 65 or Mycobacterium tuberculosis. Circ Res 2000; 86:1203-1210

(3) Kastner D, Gaffney M, Tak T. Polyarteritis nodosa and myocardial infarction. Can J Cardiol 2000; 16:515-518

(4) Koehler U, Ghahremann M, Jerrentrup A, et al. Eosinophilic myocarditis in Churg-Strauss syndrome: a rare cause of left heart decompensation with lung edema. Dtsch Med Wochenschr 2000; 125:1323-1327

(5) Takahashi K, Oharaseki T, Wakayama M, et al. Histopathological features of murine systemic vasculitis caused by Candida albicans extract: an animal model of Kawasaki disease. Inflamm Res 2004; 53:72-77

(6) del Rincon I, Escalante A. Atherosclerotic cardiovascular disease in rheumatoid arthritis. Curr Rheumatol Rep 2003; 5:278-286

(7) Wisecarver J, Jones J, Goaley T, et al. "Spontaneous" coronary artery dissection: the challenge of detection, the enigma of cause. Am J Forensic Med Pathol 1989; 10:60-62

(8) Robinowitz M, Virmani R, McAllister HAJ. Spontaneous coronary artery dissection and eosinophilic inflammation: a cause and effect relationship? Am J Med 1982; 72:923-928

(9) Lie JT, Bayardo RJ. Isolated eosinophilic coronary arteritis and eosinophilic myocarditis: a limited form of Churg-Strauss syndrome. Arch Pathol Lab Med 1989; 113:199-201

(10) Hunsaker JC III, O'Connor WN, Lie JT. Spontaneous coronary arterial dissection and isolated eosinophilic coronary arteritis: sudden cardiac death in a patient with a limited variant of Churg-Strauss syndrome. Mayo Clin Proc 1992; 67:761-766

(11) 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

(12) Martin JF, Kittas C, Triger DR. Giant cell arteritis of coronary arteries causing myocardial infarction. Br Heart J 1980; 43:487-489

(13) Paul RA, Helle MJ, Tarssanen LT. Sudden death as sole symptom of coronary arteritis. Ann Med 1990; 22:161-162

(14) Mitchinson MJ, Wight DG, Arno J, et al. Chronic coronary periarteritis in two patients with chronic periaortitis. J Clin Pathol 1984; 37:32-36

(15) Dettmeyer R, Amberg R, Varchmin-Schultheiss K, et al. Sudden cardiac death due to atypical isolated coronary arteritis? Forensic Sci Int 1998; 95:193-200

(16) Fujita M, Abe M, Itoh T, et al. Non-arthritic rheumatoid valvulitis with coronary arteritis causing myocardial infarction. Virchows Arch A Pathol Anat Histopathol 1992; 420:109-112

(17) Lie JT. Coronary vasculitis: a review in the current scheme of classification of vasculitis. Arch Pathol Lab Med 1987; 111: 224-233

(18) Cohle SD, Lie JT. Inflammatory aneurysm of the aorta, aortitis, and coronary arteritis. Arch Pathol Lab Med 1988; 112:1121-1125

(19) Burkey D, Love J, Fanning J, et al. Multiple spontaneous coronary artery dissections in a middle aged woman: support for an underlying eosinophilic arteritis predisposing to intimal disruption. Cathet Cardiovasc Diagn 1993; 30:303-305

(20) Sharma BK, Jain S, Bali HK, et al. A follow-up study of balloon angioplasty and de-novo stenting in Takayasu arteritis. Int J Cardiol 2000; 75(suppl):S147-S152

(21) Hunder GG. Epidemiology of giant-cell arteritis. Cleve Clin J Med 2002; 69(suppl):SI179-SI182

(22) Roche NE, Fulbright JW, Wagner AD, et al. Correlation of interleukin-6 production and disease activity in polymyalgia rheumatica and giant cell arteritis. Arthritis Rheum 1993; 36:1286-1294

(23) Gattenlohner S, Waller C, Ertl G, et al. NCAM(CD56) and RUNXI(AML1) are up-regulated in human ischemic cardiomyopathy and a rat model of chronic cardiac ischemia. Am J Pathol 2003; 163:1081-1090

(24) Val-Bernal JF, Mayorga M, Garcia-Alberdi E, et al. Churg-Strauss syndrome and sudden cardiac death. Cardiovasc Pathol 2003; 12:94-97

(25) Tributsch W, Rabl W, Ambach E, et al. Sudden death of a young asthmatic patient-allergic granulomatous angiitis (Churg-Strauss syndrome). Z Rechtsmed 1990; 103:385-391

(26) Pessin MS, Adelman LS, Barbas NR. Spontaneous intracranial carotid artery dissection. Stroke 1989; 20:1100-1103

Manuscript received December 2, 2004; revision accepted February 22, 2005.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal. org/misc/reprints.shtml).

Correspondence to: Philipp M. Lepper, MD, Department of Internal Medicine II, University of Ulm, Robert-Koch-Str 8, 89081 Ulm, Germany; e-mail: philipp.lepper@medizin.uniulm.de

* From the Departments of Internal Medicine II (Drs. Lepper and Koenig) and Pathology (Drs. Moller and Perner), University of Ulm, Ulm, Germany.

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COPYRIGHT 2005 Gale Group

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