Idiopathic Hypereosinophilic Vasculitis
1995; Lippincott Williams & Wilkins; Volume: 91; Issue: 11 Linguagem: Inglês
10.1161/01.cir.91.11.2851
ISSN1524-4539
AutoresM. Jakob, Manfred Ritter, J. C. Peter, E. Walter, Wolfgang Kiowski, A. Fontana, Rolf Jenni,
Tópico(s)Cardiac tumors and thrombi
ResumoHomeCirculationVol. 91, No. 11Idiopathic Hypereosinophilic Vasculitis Free AccessResearch ArticleDownload EPUBAboutView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessResearch ArticleDownload EPUBIdiopathic Hypereosinophilic Vasculitis M. Jakob, M. Ritter, J. Peter, E. Walter, W. Kiowski, A. Fontana and R. Jenni M. JakobM. Jakob From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich. , M. RitterM. Ritter From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich. , J. PeterJ. Peter From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich. , E. WalterE. Walter From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich. , W. KiowskiW. Kiowski From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich. , A. FontanaA. Fontana From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich. and R. JenniR. Jenni From the Division of Cardiology, Department of Internal Medicine, University Hospital, Zurich. Originally published1 Jun 1995https://doi.org/10.1161/01.CIR.91.11.2851Circulation. 1995;91:2851This 36-year-old male patient, at the age of 22 years, had had painless scalp swelling due to multiple aneurysms of the left temporal artery. Biopsy revealed an eosinophilic infiltration of all three wall layers with fresh fibrinoid necrosis beside old fibrous scars. An idiopathic blood eosinophilia was noted; no treatment was initiated. In the present case, the 36-year-old patient presented with dyspnea at rest. The ECG showed evidence of prior inferior infarction. Transesophageal echocardiography found left ventricular (LV) enlargement with biplanar ejection fraction of 20% as well as dilatation of the right ventricle and the atria. LV muscle mass was increased (245 g/m2; normal, 134 g/m2). The epicardial coronary arteries showed grotesque aneurysmal dilatation. Intravascular thrombosis of the left and right main stem with reticular revascularization could be clearly identified (Fig 1). Ultrasonic myocardial texture was normal, without signs of focal granulomatosis, whereas endocardial echo density was enhanced, indicating endocardial fibrosis. Right ventricular endomyocardial biopsy yielded interstitial scars in the myocardium. However, there was no infiltration with eosinophilic leukocytes. Angiography visualized proximal obliteration of the left anterior descending and right coronary arteries. The left circumflex artery was subtotally obliterated (Figs 2 and 3). Aortography revealed a normal abdominal aorta and intestinal arteries but a stenosis of the arteria hepatica propria. The small vessels of the inferior spleen were not patent. Laboratory tests found hypereosinophilia (48%) and elevated IgE. There was no evidence of parasitosis, atopic disease (eg, asthma), or neoplastic or connective tissue disease. Persistent eosinophilia from various causes may be associated with the so-called hypereosinophilic syndrome, comprising endomyocardial fibrosis, valvular lesions, mural thrombus formation, cardiomegaly due to infiltration of the myocardium with eosinophils, and pericardial effusion.123 Furthermore, the patients have asthma and gastrointestinal and nervous system dysfunction. In contrast, the patient presented here has no signs of widespread infiltration of organs with eosinophils but shows systemic vasculitis with eosinophilia and fibrinoid necrosis, leading to aneurysm formation. The clinical picture is distinct from Churg-Strauss syndrome, periarteritis nodosa, and juvenile arteritis temporalis, all of which are also occasionally described to manifest at the coronary vessels and to be associated with blood eosinophilia.45 However, precise nosological classification of the systemic eosinophilic vasculitis in our patient is not possible because of absence of organ involvement and negative tests for autoantibodies, eg, anti-neutrophil cytoplasmic antibodies. The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Lukes Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine. Circulation encourages readers to submit cardiovascular images to Dr Hugh A. McAllister, Jr, St Luke's Episcopal Hospital and Texas Heart Institute, 6720 Bertner, MC 4-265, Houston, TX 77030. Download figureDownload PowerPoint Figure 1. (Left) Transesophageal oblique echocardiographic view of the aortic root (AO) and adjacent structures at the level of the coronary arteries. A large aneurysm of the left circumflex coronary artery (bold arrow) with an endoluminal thrombus is visualized to the left of the ectatic left main stem. Aneurysmal dilatation of the right coronary artery (small arrow) is depicted anterior to the aortic root. LA indicates left atrium.Download figureDownload PowerPoint Figure 2. (Center) Left anterior oblique projection of contrast dye injection into the occluded right coronary artery visualizes a large proximal aneurysm.Download figureDownload PowerPoint Figure 3. (Right) Right anterior oblique projection of the left coronary artery. The left anterior descending artery is occluded. The left circumflex artery (CX) as well as a large intermediate branch (IB) show grotesque aneurysmal dilatation.FootnotesCorrespondence to Markus Jakob, MD, Department of Internal Medicine, University Hospital, CH-8091, Zurich, Switzerland. References 1 Fauci A, Harley J, Roberts W, Ferrans V, Gralnick H, Bjornson B. The idiopathic hypereosinophilic syndrome. Ann Intern Med.1982; 97:78-92. CrossrefMedlineGoogle Scholar2 van den Bosch J, Wagenaar S, Westermann C. Asthma, eosinophilic pleuropneumonia, and pericarditis without vasculitis. Thorax.1986; 41:571-572. CrossrefMedlineGoogle Scholar3 Jolobe O, Melnick S. Asthma, pulmonary eosinophilia, and eosinophilic pericarditis. Thorax.1983; 38:690-691. CrossrefMedlineGoogle Scholar4 Hirakawa Y, Koyanagi S, Matsumoto T, Takeshita A, Nakamura M. A case of variant angina associated with eosinophilia. Am J Med.1989; 87:472-474. CrossrefMedlineGoogle Scholar5 Hertzman P, Maddoux G, Sternberg E, Heyes MP, Mefford IN, Kephart GM, Gleich GJ. Repeated coronary artery spasm in a young woman with the eosinophilia-myalgia syndrome. JAMA.1992; 267:2932-2934. CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Silbiger J (2013) Echocardiographic Examination of the Posterior Atrioventricular Groove, Echocardiography, 10.1111/echo.12438, 31:2, (223-233), Online publication date: 1-Feb-2014. Harzy T, Allali F, Amine B, Rahmouni R and Hajjaj-Hassouni N (2005) Manifestations cardiaques du syndrome hyperéosinophilique essentiel, La Revue de Médecine Interne, 10.1016/j.revmed.2004.09.023, 26:5, (386-392), Online publication date: 1-May-2005. June 1, 1995Vol 91, Issue 11 Advertisement Article InformationMetrics Copyright © 1995 by American Heart Associationhttps://doi.org/10.1161/01.CIR.91.11.2851 Originally publishedJune 1, 1995 KeywordsCardiovascular Imagescoronary aneurysmvasculitis Advertisement
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