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Table 2 Clinical manifestations and prognosis of previously reported CAEBV patients with coronary artery lesions

From: Chronic active Epstein–Barr virus infection manifesting as coronary artery aneurysm and uveitis

Pt

Sex

Onset age (years)

IM-like symptoms

Coronary artery lesions

Other cardiovascular complications

Other organ manifestations

Occurrence age (years)

Affected branch

Coronary diameter

Large-vessel arteritis

Myocarditis

Others

1

M

4

L, H

4

NA

NA

No

No

No

Interstitial pneumonitis, Sjögren syndrome

2

F

5

L, H

5

NA

NA

Abdominal aortic aneurysm

No

No

Interstitial pneumonitis

3

M

2

F, L, H

2

NA

NA

Dilation of the sinus of Valsalva

No

No

Interstitial pneumonitis

4

F

6

F, L, H

6

NA

NA

No

No

Pericarditis

Interstitial nephritis

5

F

5

F, H

5

NA

NA

Dilation of the sinus of Valsalva

No

Pericarditis

No

6

F

2

F, H

4

LCA

4 mm

No

No

No

Gastrointestinal tract, hypersensitivity to mosquito bites, hydroa vacciniforme

7

M

5

F, H

8

LCA

5 mm

No

No

No

No

8

M

6

F, H

10

LCA

4.5 mm

No

No

No

No

9

F

9

F, H

18

LCA, RCA

4 mm in RCA, 4 mm in LCA

No

No

No

No

10

M

11

H

14

LCA, RCA

NA

No

No

PAH, junctional ectopic tachycardia

No

11

F

10

F, H

10

LCA, RCA

NA

Dilation of bilateral common carotid and subclavian arteries, abdominal aorta and major branches, and bilateral common iliac arteries

No

No

No

12

M

2

F, H, L

2

LCA, RCA

6 mm in RCA, 3 mm in LCA

No

No

No

No

13

F

7

F, H, L

7

NA

NA

No

No

Pericardial effusion

No

14

M

2

F, H, L

2

LAD, LCX

6 mm in LMCA

No

No

No

No

15

F

5

F, H

5

LCA, RCA

8 mm in RCA, 5 mm in LCA

38 mm in the sinus of Valsalva, thoracic and abdominal aortic aneurysms

No

No

No

16

M

6

F, H

8

NA

NA

No

No

No

No

17

F

NA

NA

NA

LCA, RCA

NA

No

No

No

No

18

F

16

F, H, L

16

RCA (multiple stenoses and dilation)

NA

Aneurysms involving the aorta and its major branches, and bilateral common iliac arteries

No

No

No

19

F

6

F, H, L

6

LMCA, RCA

5 mm in LMCA, 8.2 mm in RCA

No

No

No

No

20

M

5

NA

9

LAD, RCA

NA

Dilatation of aortic sinus, minor dilatation of abdominal aortic stem and the distal section of superior mesenteric artery

No

PAH, cardiac insufficiency

Skin ulcers

Pt

Sex

Onset age (years)

IM-like symptoms

EBV status

Therapies—HSCT

Prognosis

Reports

Cell type

EBV copies (in PB)/EBERs

Abnormal EBV antibodies

1

M

4

L, H

T

EBERs (+)

Yes

No

Died (streptococcus pneumoniae) within 36 months from onset

Nakagawa et al. [25]

2

F

5

L, H

T

EBERs (+)

Yes

No

Died (respiratory failure) within 13 months from onset

Nakagawa et al. [25]

3

M

2

F, L, H

NA

EBV-DNA in cardiac tissues

Yes

No

Died within 5 years from onset

Kikuta et al. [26]

4

F

6

F, L, H

NA

EBV-DNA in cardiac and aortic tissues

Yes

No

Died within 5 years from onset

Kikuta et al. [26]

5

F

5

F, H

NA

EBV-DNA in cardiac tissues

Yes

No

Died within 5 years from onset

Kikuta et al. [26]

6

F

2

F, H

NK

3 × 103 copies/mL

No

Yes

Alive and disease free

Muneuchi et al. [12]

7

M

5

F, H

T

3 × 103 copies/mL

Yes

Yes

Died at 14 years

Muneuchi et al. [12]

8

M

6

F, H

T

5 × 104 copies/mL

Yes

Yes

Alive and disease free

Muneuchi et al. [12]

9

F

9

F, H

γδT

9 × 102 copies/mL

Yes

No

Died at 18 years

Muneuchi et al. [12]

10

M

11

H

NA

8.2 × 104 copies/μg DNA

Yes

Yes

Died (septic shock and multiple organ failure) within 13 days after transplantation

Fukuda et al. [27]

11

F

10

F, H

T

EBERs (+)

Yes

No

Died (respiratory failure due to diffuse alveolar damage)

Murakami et al. [28]

12

M

2

F, H, L

T

EBV-DNA in PBMC

Yes

NA

NA

Kikuta et al. [29]

13

F

7

F, H, L

T

EBV-DNA in pericardial effusion

Yes

Yes

Died (failure of umbilical cord blood transplantation)

Toubo et al. [30]

14

M

2

F, H, L

T

NA

Yes

No

NA

Kobayashi et al. [31]

15

F

5

F, H

T

EBERs (+)

Yes

No

Died (acute respiratory failure) within 13 months from onset

Nakagawa et al. [14]

16

M

6

F, H

T

NA

Yes

NA

NA

Ohga et al. [32]

17

F

NA

NA

NA

NA

NA

Yes

NA

Nishimura et al. [33]

18

F

16

F, H, L

NA

2.8 × 104 copies/mL

NA

No

NA

Jiang et al. [34]

19

F

6

F, H, L

NA

3.72 × 107 copies/mL

NA

No

NA

Xie et al. [35]

20

M

5

NA

NA

4.53 × 103 copies/mL in plasma, EBERs (+)

Yes

No

NA

Ba et al. [36]

  1. NA not available, F fever, H hepatosplenomegaly, L lymphadenopathy, LAD left anterior descending artery, LCA left coronary artery, LCX left circumflex artery, LMCA left main coronary artery, RCA right coronary artery, PAH pulmonary arterial hypertension, PB peripheral blood, PBMC peripheral blood mononuclear cell