Circulation. 1995;91:2851
(Circulation. 1995;91:2851.)
© 1995 American Heart Association, Inc.
Idiopathic Hypereosinophilic Vasculitis
M. Jakob, MD;
M. Ritter, MD;
J. Peter, MD;
E. Walter, MD;
W. Kiowski, MD;
A. Fontana, MD;
R. Jenni, MD
From the Division of Cardiology, Department of Internal Medicine,
University Hospital, Zurich.
Correspondence to Markus Jakob, MD, Department of Internal Medicine,
University Hospital, CH-8091, Zurich, Switzerland.
Key Words: Cardiovascular Images • vasculitis • coronary aneurysm
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Introduction
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This
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.
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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.
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Figure 2. (Center) Left anterior oblique
projection of contrast dye injection into the occluded right coronary
artery visualizes a large proximal aneurysm.
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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.
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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.1 2 3
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.4 5
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.
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Footnotes
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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.
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References
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Fauci A, Harley J, Roberts W, Ferrans V,
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Jolobe O, Melnick S. Asthma,
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Hirakawa Y, Koyanagi S, Matsumoto T,
Takeshita A, Nakamura M. A case of variant angina associated
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[Order article via Infotrieve]
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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. [Abstract]
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Introduction
References