Circulation. 2000;101:e69-e70
(Circulation. 2000;101:e69.)
© 2000 American Heart Association, Inc.
Circulation Electronic Pages |
Cardiac Vasculitis in Henoch-Schönlein Purpura
Abdulfatah Osman, MD;
Charles J. McCreery, MD
From the Division of Cardiology, University of Texas, Galveston.
Correspondence to Charles J. McCreery, MD, FACC, University of Texas, 301 University Blvd, Galveston, TX 77555-0553. E-mail charles.mccreery{at}utmb.edu
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Introduction
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 Top
 Introduction
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A63-year-old man
presented with a 1-week history of bloody diarrhea,
abdominal
pain, nausea, arthralgias, and fatigue. Physical examination
revealed
an acutely ill patient with a distended, tender abdomen.
A purpuric
skin rash was noted on the extremities and trunk
(Figure 1
). Laboratory tests showed leukocytosis,
proteinuria,
and elevated creatinine. A skin biopsy
revealed small-vessel
neutrophilic vasculitis.
Immunofluorescence was positive for
multifocal IgA
deposits along the walls of dermal vessels (Figure
2
). High-dose prednisone and azathioprine
were started. On hospital
day 4, the patient developed slow junctional
rhythm with hypotension
requiring transvenous ventricular
pacing. Serum cardiac troponin
T was elevated. Sinus rhythm never
recovered, and ectopic low
atrial rhythm predominated (Figure 3
). The subsequent course
was marked by
worsening renal failure, noncardiogenic pulmonary
edema, and
respiratory failure. The patient died despite maximal
supportive care.
At autopsy, the heart showed confluent ecchymoses
involving the entire
right atrium (Figure 4
). Multiple
sections
from the atrium, including the area of the sinoatrial node,
showed
neutrophilic myocarditis and diffuse small-vessel
leukocytoclastic
vasculitis with fibrinoid necrosis (white arrow) and
interstitial
hemorrhages (black arrow) (Figure 5
). The cardiac chambers and
great
vessels were spared. Other findings included intestinal
serosal
hemorrhages, bronchial mucosal ecchymoses, and focal
segmental
glomerulonephritis positive for IgA
deposits.
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Figure 2. Immunofluorescence examination
shows strong positivity for IgA deposits in walls of dermal small
vessels.
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Figure 4. Exposed endocardial surface of right atrium and
ventricle shows diffuse, confluent, subendocardial hemorrhages
involving entire right atrium. Ecchymotic endocardium is sharply
demarcated at tricuspid ring and at inlets of vena cava.
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Figure 5. Hematoxylin-eosin–stained sections from right
atrium showing necrotizing leukocytoclastic vasculitis of a small
atrial vessel. Neutrophilic infiltrate and nuclear debris are seen in
and around necrotic vessel (white arrow). Interstitial
hemorrhages are present in background (black arrow).
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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 Luke’s Episcopal
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 Ave, MC1-267, Houston, TX 77030.
