IMAGES THAT TEACH
Large-vessel vasculitis with persistent exertional
dyspnea in a 75-year-old woman
Shiro Miura, MD, MSc,a Masanao Naya, MD, PhD,b Osamu Manabe, MD,PhD,c and Takehiro Yamashita, MD, PhD, FACCaabc

Department of Cardiology, Hokkaido Ohno Memorial Hospital, Sapporo, Japan
Department of Cardiovascular Medicine, Hokkaido University Graduate School of Medicine,Sapporo, Japan
Department of Radiology, Jichi Medical University Saitama Medical Center, Saitama, Japan

Received Jul 13, 2021; accepted Jul 14, 2021
doi:10.1007/s12350-021-02753-0

AbbreviationsCMD
Coronary microvascular dysfunctionCT
Computed tomography
FDG
Fluorodeoxyglucose
MFR
Myocardial flow reserve

CASE SUMMARY
A 75-year-old woman presented with low-grade
fever, and pain and numbness in her upper extremities
that worsened on exertion, for over three months,(Raynaud’s phenomenon) (Figure 1). She also had
persistent exertional dyspnea. Her electrocardiographic

Reprint requests: Shiro Miura, MD, MSc, Department of Cardiology,
Hokkaido Ohno Memorial Hospital, 2-1-16-1 Miyanosawa, Nishiku, Sapporo063-0052, Japan; s.miura@ohno-kinen.or.jp
J Nucl Cardiol
1071-3581/$34.00
Copyright Ó 2021 American Society of Nuclear Cardiology.

PETRPR

Positron emission tomography
Rate–pressure productand transesophageal echocardiographic findings were
within the normal ranges. Computed tomography (CT)angiography revealed multiple stenoses in both midsubclavian arteries and occlusion from the axillary to thebrachial arteries bilaterally (Figure 2). Subsequent invasive coronary angiography demonstrated intermediateMiura et al
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Figure 1. The left hand turned pale after repetitive hand grip (A), then returned to normal when
warmed (B), in contrast to the normal right hand.

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stenotic lesions at the ostium of the right and left
coronary arteries (Figure 3). 18F-fluorodeoxyglucose(FDG) positron emission tomography (PET)/CT
revealed an increased FDG uptake in the thoracoabdominal aorta and along the carotid and subclavian
arteries (Figure 4A). Laboratory examination showed awhite blood cell count of 8200/mm3 and erythrocyte
sedimentation rate of 81 mmh. Blood cultures andserologic tests were negative for hepatitis B, hepatitis C,
human immunodeficiency virus, and syphilis. The abovefindings were suggestive of Takayasu’s arteritis. Her
stress/rest perfusion 13N-ammonia PET/CT demonstrated (Figure 5A) a significantly low global

Figure 2. Three-dimensional reconstruction of the greatarteries by computed tomography demonstrates stenoses inboth the mid-subclavian arteries (blue arrowheads) and total
long occlusion from the axillary to brachial arteries (redarrowheads) in both arms, without any significant stenosis of
the thoracoabdominal aorta. Focal ostial stenosis of the right
renal artery (yellow arrow) is evident.

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Figure 3. Invasive coronary angiography (A) demonstrates unobstructed epicardial coronary
arteries with intermediate ostial stenoses (yellow arrows) in the left (A, left) and right coronary
arteries (A, right), assessed at 42% and 31%, respectively, through qualitative comparative analysis.
Digital subtraction angiography (B) confirms significant stenosis (blue arrowheads) of the bilateralsubclavian arteries and occlusion from the axillary to brachial arteries bilaterally (red arrowheads).

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myocardial flow reserve (MFR) of 1.82. However,myocardial perfusion imaging appeared normal, suggesting coronary microvascular dysfunction (CMD). She
was treated with oral prednisone. Inflammatory parameters normalized within three weeks, and the numbness
and pain in her arms disappeared. On repeated FDGPET/CT, no remarkable uptake along the aortic wall andsubclavian arteries was noted (Figure 4B). Three months
after the initiation of corticosteroid therapy, she still
complained of dyspnea on exertion. A repeat 13Nammonia PET/CT (Figure 5B) revealed a low global
MFR of 2.09. These findings imply that conventional
corticosteroid treatment may not improve angina and
CMD over the short-term. CMD has been described insimilar chronic inflammatory conditions, such as systemic lupus erythematosus, where prolonged vascularinflammation contributes to endothelial dysfunction andreduced MFR.1 However, this is the first report that

Figure 4. Initial 18F-fluorodeoxyglucose (FDG) positronemission tomography (PET)/CT demonstrates increased FDG
uptake in the thoracoabdominal aorta, and along the carotidand subclavian arteries (red arrows), with a single horizontal
slice at the pulmonary trunk level (A). In contrast, theenhanced FDG uptake (white arrowheads) almost disappeared
after the 3-week oral corticosteroid therapy (B).

Figure 5. Comparison of the initial (A) and repeat (B, after 3 months of corticosteroid therapy)ATP-induced stress and rest 13N-ammonia PET/CT. The initial global myocardial blood flow(MBF) at stress and rest were 2.58 and 1.45 mLmin-1g-1, respectively; the initial and follow-up
myocardial flow reserve (MFR) were 1.82 (rate–pressure product [RPP]-corrected MFR, 1.56) (A,left) and 2.09 (RPP-corrected MFR, 1.45), respectively, with the stress MBF unchanged at 2.58mLmin-1g-1 (B, left). These findings were similar in the 17-segment between the initial and followup PET examinations (A, right and B, right). Myocardial perfusion imaging in the short axis (SA),vertical long axis (VLA), and horizontal long axis (HLA) showed no significant reduction in tracer
uptake during stress and rest phases in both studies (A, bottom and B, bottom).

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associates large-vessel vasculitis with CMD, as evidenced by perfusion PET.

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Reference

Disclosures

1. Behar JM, Bratis K, Clapp B, Nagel E. Refractory ischaemic chest
pain caused by microvascular coronary dysfunction in a large vesselvasculitis. Eur Heart J Cardiovasc Imaging 2016;17:702

The authors indicate that they have no financial conflictof interest.

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