Table 4 Clinical information in each case of Ext1/Ext2–associated MN.

From: Prevalence of neural epidermal growth factor-like 1- and exostosin 1/exostosin 2-associated membranous nephropathy: a single-center retrospective study in Japan

Case

Clinical diagnosis

Age

Sex

Proteinuria (g/day)

Serum creatinine (mg/dl)

Serum albumin (g/dl)

Complications

ANA/dsDNA/others

C3/C4/CH50

IHC of IgG4

Immunosuppressive therapy

1

Primary

55

Female

0.8

0.53

2.9

None

neg/NA/NA

Normal

neg

None

2

Primary

63

Male

2.1

1.00

3.2

None

neg/NA/NA

Normal

pos

Prednisolone + Cyclosporine

3

Primary

62

Female

1.3

0.48

2.5

None

neg/NA/NA

Normal

neg

Prednisolone

4

Primary

59

Male

2.5

1.16

4.3

None

neg/NA/NA

Normal

neg

None

5

Primary

53

Male

1.1

1.35

4.0

None

NA/NA/NA

NA

neg

Prednisolone

6

Primary

49

Male

0.6

0.73

3.8

None

320/neg/neg

Normal

neg

None

7

Primary

29

Female

6.1

0.51

2.0

None

320/neg/neg

Normal

pos

Prednisolone

8

Primary #1

49

Male

2.1

0.68

3.0

None

neg/neg/neg

Normal

pos

Prednisolone

9

Primary#2

69

Female

8.2

1.07

1.5

None

neg/NA/NA

Normal

pos

Prednisolone + Cyclosporine

10

Secondary

25

Male

0.2

0.70

4.3

Hepatitis B

neg/NA/NA

Normal

neg

None

11

Secondary

37

Male

1.5

0.68

2.2

Hepatitis B

neg/NA/NA

Normal

neg

Interferon

12

Secondary

65

Male

6.7

0.95

1.7

Prostate cancer

Ulcerative colitis

Guillain–Barre syndrome

neg/neg/neg

Normal

neg

Prednisolone

13

Secondary

35

Female

3.3

0.58

2.2

Thyroiditis

1280/neg/neg

Normal

neg

Prednisolone

14

Secondary

67

Male

2.1

0.83

3.5

Lung cancer

neg/NA/NA

Normal

neg

None

15

Secondary

46

Male

1.2

0.80

3.5

Lupus

320/neg/RNP, ssDNA,SS-A

33/9/ < 10

neg

Prednisolone + Tacrolimus

16

Secondary

59

Female

1.8

0.72

2.7

Lupus + scleroderma

640/neg/ssDNA

34/14/5

neg

Prednisolone + Mycophenolate mofetil

17

Secondary

52

Male

0.5

0.50

4.0

Lupus

1280/neg/ssDNA

Normal

neg

Prednisolone

  1. ANA antinuclear antibody, neg negative, pos positive, NA not available, ssDNA single strand DNA, dsDNA double strand DNA, #1 enhanced staining of PLA2R was also found, #2 enhanced staining of THSD7A was also found.