Table 3 Clinical characteristics of 11 cases of TAFRO syndrome with kidney biopsy

Case no./Ref.

Age/sex

UP

Alb (g/dL)

Cr (mg/dL)

CRP (mg/dL)

VEGF/IL-6 (pg/mL)

1st Therapy/ response

2nd Therapy/ response

Kidney biopsy findings

Thrombi

Endothelial swelling

Endocapillary proliferation

Mesangiolysis

Mesangial proliferation

Double contours of GBM

Glomerular Ig deposition by IF

Subendothelial lesions by EM

Electron dense deposits

Diagnosis by authors

1/ [4]

38 / M

n.d.

3

2.59

11.18

4420 / n.d.

PSL / good

-

n.d.

n.d.

n.d.

n.d.

Yes

Yes

Yes

n.d.

n.d.

MPGN-like

2/ [5]

55 / F

0.54 (g/g・Cr)

2.7

2.1

4.1

464 / 11.7

Pulse steroid / good

-

n.d.

n.d.

n.d.

n.d.

Yes

Yes

Yes

n.d.

No

MPGN-like

3/ [6]

76 / F

0.30 (g/day)

1.2

3.02

16

1350 / 49.2

PSL /good

-

n.d.

n.d.

Yes

n.d.

Yes

Yes

No

Yes

No

MPGN-like

4/ [7]

79 / F

2.65 (g/g・Cr)

2.4

1.85

3.9

15.6 / 3.76

Pulse steroid, PE / bad

RTX / good

n.d.

n.d.

No

Yes

No

Yes

No

Yes

n.d.

TMA-like

5/ [8]

70 / M

0.33 (g/day)

2.5

1.28

9.85

126 / 33

Pulse steroid / good

-

No

Yes

No

Yes

No

Yes

No

Yes

No

MPGN-like

6/ [9]

61 / F

n.d.

2.8

2.14

23.12

n.d. / 722.6

Pulse steroid, TCZ, RTX / good

-

n.d.

Yes

n.d.

Yes

Yes

Yes

No

n.d.

n.d.

MPGN-like

7/ [10]

80 / F

0.41 (g/day)

2.8

1.17

7.3

454 / 21.6

PSL /bad

TCZ / good

No

Yes

Yes

No

No

Yes

Yes

Yes

No

TMA-like

8/ [11]

51 / F

0.52 (g/g・Cr)

2.5

1.03

4.58

198 / 21.2

Pulse steroid / good

-

n.d.

Yes

No

Yes

No

Yes

No

n.d.

n.d.

TMA-like

9/ [12]

84 / M

0.30 (g/day)

2.4

2.31

8.3

177 / 12.3

PSL, PE /bad

TCZ / good

No

Yes

Yes

Yes

No

No

No

Yes

No

TMA-like

10/ [13]

54 / F

3.2 (g/g・Cr)

2.4

1.11

7

n.d. / 8.2

PSL /good

-

No

Yes

No

No

No

No

No

Yes

No

Endothelial injury

our case

48 / F

1.57 (g/g・Cr)

2.6

1.32

18.33

494 / 166

Pulse steroid /bad

TCZ / good

No

Yes

Yes

Yes

No

Yes

No

Yes

No

TMA-like