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Clinical and Experimental Nephrology

, Volume 15, Issue 4, pp 493–503 | Cite as

Japan Renal Biopsy Registry: the first nationwide, web-based, and prospective registry system of renal biopsies in Japan

  • Hitoshi Sugiyama
  • Hitoshi YokoyamaEmail author
  • Hiroshi Sato
  • Takao Saito
  • Yukimasa Kohda
  • Shinichi Nishi
  • Kazuhiko Tsuruya
  • Hideyasu Kiyomoto
  • Hiroyuki Iida
  • Tamaki Sasaki
  • Makoto Higuchi
  • Motoshi Hattori
  • Kazumasa Oka
  • Shoji Kagami
  • Michio Nagata
  • Tetsuya Kawamura
  • Masataka Honda
  • Yuichiro Fukasawa
  • Atsushi Fukatsu
  • Kunio Morozumi
  • Norishige Yoshikawa
  • Yukio Yuzawa
  • Seiichi Matsuo
  • Yutaka Kiyohara
  • Kensuke Joh
  • Takashi TaguchiEmail author
  • Hirofumi Makino
  • Committee for Standardization of Renal Pathological Diagnosis and Working Group for Renal Biopsy Database, Japanese Society of Nephrology, Tokyo, Japan
Original Article

Abstract

Background

The Committee for the Standardization of Renal Pathological Diagnosis and the Working Group for Renal Biopsy Database of the Japanese Society of Nephrology started the first nationwide, web-based, and prospective registry system, the Japan Renal Biopsy Registry (J-RBR), to record the pathological, clinical, and laboratory data of renal biopsies in 2007.

Methods

The patient data including age, gender, laboratory data, and clinical and pathological diagnoses were recorded on the web page of the J-RBR, which utilizes the system of the Internet Data and Information Center for Medical Research in the University Hospital Medical Information Network. We analyzed the clinical and pathological diagnoses registered on the J-RBR in 2007 and 2008.

Results

Data were collected from 818 patients from 18 centers in 2007 and 1582 patients from 23 centers in 2008, including the affiliated hospitals. Renal biopsies were obtained from 726 native kidneys (88.8%) and 92 renal grafts (11.2%) in 2007, and 1400 native kidneys (88.5%) and 182 renal grafts (11.5%) in 2008. The most common clinical diagnosis was chronic nephritic syndrome (47.4%), followed by nephrotic syndrome (16.8%) and renal transplantation (11.2%) in 2007. A similar frequency of the clinical diagnoses was recognized in 2008. Of the native kidneys, the most frequent pathological diagnosis as classified by pathogenesis was immunoglobulin (Ig) A nephropathy (IgAN) both in 2007 (32.9%) and 2008 (30.2%). Among the primary glomerular diseases (except IgAN), membranous nephropathy (MN) was the most common disease both in 2007 (31.4%) and 2008 (25.7%).

Conclusions

In a cross-sectional study, the J-RBR has shown IgAN to be the most common disease in renal biopsies in 2007 and 2008, consistent with previous Japanese studies. MN predominated in the primary glomerular diseases (except for IgAN). The frequency of the disease and the clinical and demographic correlations should be investigated in further analyses by the J-RBR.

Keywords

Glomerulonephritis Tubulointerstitial disorder Renal vascular disease Renal grafts National registry 

Introduction

There has been no national registry of renal biopsies in Japan. The Committee for the Standardization of Renal Pathological Diagnosis and the Working Group for Renal Biopsy Database in the Japanese Society of Nephrology established the first nationwide, web-based, and prospective registry system, the Japan Renal Biopsy Registry (J-RBR), to record pathological, clinical, and laboratory data regarding all renal biopsies performed in 2007.

To date, the epidemiological and clinical data of renal diseases are available from nationwide registries of renal biopsies from the United Kingdom [1], Italy [2], Denmark [3], Spain [4], the Czech Republic [5], and Australia [6]. The role of a renal biopsy registry has been recently encouraged [7]. In Japan, several surveys were temporarily conducted for patients with restricted renal diseases, including primary glomerulonephritis [8], idiopathic membranous nephropathy (MN) [9], and immunoglobulin (Ig) A nephropathy (IgAN) [10]. However, there has been no web-based, nationwide, or prospective registry system of overall renal biopsies in Japan. The aim of the current study was to provide data to investigate the epidemiology and frequency of renal diseases with a histological diagnosis for patients registered in 2007 and 2008 on the J-RBR.

Subjects and methods

Registry system and patients

The researchers on the Committee for the Standardization of Renal Pathological Diagnosis and the Working Group for Renal Biopsy Database in the Japanese Society of Nephrology participated in this study. The report includes the data from patients on the J-RBR, registered prospectively from January to December of 2007 and 2008. Patient data including age, gender, laboratory data, and the clinical and pathological diagnoses were electronically recorded at each institution and registered on the web page of the J-RBR utilizing the system of Internet Data and Information Center for Medical Research (INDICE) in the University Hospital Medical Information Network (UMIN). The ethical committee of the Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences comprehensively approved the study, and a local committee of participating centers and their affiliated hospitals individually approved the study. Written informed consent was obtained from the patients at the time of biopsy or before participation in the study. The J-RBR is registered to the Clinical Trial Registry of UMIN (registered number UMIN000000618) and is available in Japanese and English.

Clinical or renal histopathological diagnosis and laboratory data

Three classifications, clinical diagnosis, histological diagnosis by pathogenesis, and histological diagnosis by histopathology, were selected for each case (Supplementary Table) from the J-RBR. The classification of clinical diagnoses was determined as follows: acute nephritic syndrome, rapidly progressive nephritic syndrome, recurrent or persistent hematuria, chronic nephritic syndrome, nephrotic syndrome, renal disorder with metabolic disease, renal disorder with collagen disease or vasculitis, hypertensive nephropathy, inherited renal disease, acute renal failure, drug-induced nephropathy, renal transplantation, and others. The definitions of the former five clinical diagnoses were based on the clinical syndromes and glomerular histopathology in the classification of glomerular diseases [11]. Acute nephritic syndrome was defined as a syndrome characterized by the abrupt onset of hematuria, proteinuria, hypertension, decreased glomerular filtration, and edema. Rapidly progressive nephritic syndrome was defined as an abrupt or insidious onset of hematuria, proteinuria, anemia, and rapidly progressing renal failure. Recurrent or persistent hematuria included the insidious or abrupt onset of gross or microscopic hematuria with little or no proteinuria and no evidence of other features of nephritic syndrome. Chronic nephritic syndrome was defined as slowly developing renal failure accompanied by proteinuria, hematuria, with or without hypertension. Nephrotic syndrome was defined as massive proteinuria >3.5 g/day and hypoalbuminemia of <3 g/dL of serum albumin with or without edema or hypercholesteremia.

The renal histological diagnosis is classified either according to pathogenesis (A) or by histopathology (B) as follows: (A) primary glomerular disease (except IgAN), IgAN, purpura nephritis, lupus nephritis, myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA)-positive nephritis, protein 3 (PR3)-ANCA-positive nephritis, anti-glomerular basement membrane antibody nephritis, hypertensive nephrosclerosis, thrombotic microangiopathy, diabetic nephropathy, amyloid nephropathy, Alport syndrome, thin basement membrane disease, infection-related nephropathy, transplanted kidney, and others; (B) minor glomerular abnormalities, focal and segmental glomerulosclerosis (FSGS), MN, mesangial proliferative glomerulonephritis, endocapillary proliferative glomerulonephritis, membranoproliferative glomerulonephritis (MPGN) (type I, III), dense deposit disease, crescentic and necrotizing glomerulonephritis, sclerosing glomerulonephritis, nephrosclerosis, acute interstitial nephritis, chronic interstitial nephritis, acute tubular necrosis, transplanted kidney, and others. IgAN (Berger disease) was separated from primary glomerular diseases on the basis of basic glomerular alterations in the classification of glomerular diseases [11]. Clinical data, including urinalysis, daily proteinuria, serum creatinine concentrations, total protein, albumin, and total cholesterol values were also recorded, but only the frequency of the disease is described here.

Statistics

Data were expressed as mean ± SD as appropriate. Statistical analyses were performed using the JMP software program, version 8 (SAS Institute Inc., Cary, NC, USA).

Results

Baseline characteristics of registered biopsies

Data were collected from 818 patients from 18 centers in 2007 and 1582 patients from 23 centers in 2008, including the affiliated hospitals. Renal biopsies were obtained from 726 native kidneys (88.8%) and 92 renal grafts (11.2%) in 2007 and 1400 native kidneys (88.5%) and 182 renal grafts (11.5%) in 2008 (Table 1). The average age of the patients was 44.6 ± 20.7 years of age in 2007 and 44.2 ± 21.1 years of age in 2008. A higher number of male patients than female patients were registered in both years (male patients 52.6% in 2007 and 53.8% in 2008). The distribution of the total number of renal biopsies according to age and gender are presented in Fig. 1, and reveals a different age and gender distribution in native kidneys and renal grafts.
Table 1

Number of participating renal centers and registered renal biopsies on the Japan Renal Biopsy Registry (J-RBR) in 2007 and 2008

Year

2007

2008

Total

Renal centers

18

23

23

Total biopsies

818

1582

2400

 Average age (y)

44.6 ± 20.7

44.2 ± 21.1

44.4 ± 21.0

 Male

430

851

1281

 Female

388

731

1119

Native kidneys

726

1400

2126

 Average age (y)

45.2 ± 21.4

44.8 ± 22.0

44.9 ± 21.5

 Male

378

751

1129

 Female

348

649

997

Renal grafts

92

182

274

 Average age (y)

40.5 ± 13.5

39.4 ± 16.3

39.8 ± 15.4

 Male

52

100

152

 Female

40

82

122

Fig. 1

Distribution of age ranges and gender in total renal biopsies (a), native kidneys (b), and renal grafts (c) in the combined data of 2007 and 2008

The frequency of clinical diagnoses

The clinical diagnosis and renal histological diagnosis as classified by pathogenesis and by histopathology were determined for each biopsy. A clinical diagnosis of chronic nephritic syndrome was the most frequent, followed by nephrotic syndrome and renal transplantation in 2007, which was similar in 2008 (Table 2). In native kidneys, the majority of the cases corresponded to chronic nephritic syndrome, followed by nephrotic syndrome and recurrent or persistent hematuria or renal disorder with collagen disease or vasculitis in 2007 (Table 2). Similar frequencies of chronic nephritic syndrome, nephrotic syndrome and renal disorder with collagen disease or vasculitis were observed in 2008 (Table 2).
Table 2

Frequency of classification of clinical diagnoses

Classification

2007

2008

Total

n

%

n

%

n

%

Chronic nephritic syndrome

388

47.4

768

48.5

1156

48.2

Nephrotic syndrome

138

16.9

259

16.4

397

16.5

Renal transplantation

92

11.2

182

11.5

274

11.4

Renal disorder with collagen disease or vasculitis

41

5.0

87

5.5

128

5.3

Rapidly progressive nephritic syndrome

33

4.0

80

5.1

113

4.7

Recurrent or persistent hematuria

41

5.0

33

2.1

74

3.1

Renal disorder with metabolic syndrome

29

3.5

46

2.9

75

3.1

Hypertensive nephropathy

14

1.7

30

1.9

44

1.8

Acute nephritic syndrome

15

1.8

20

1.3

35

1.5

Acute renal failure

7

0.9

13

0.8

20

0.8

Drug-induced nephropathy

3

0.4

11

0.7

14

0.6

Inherited renal disease

5

0.6

8

0.5

13

0.5

Others

12

1.6

45

2.8

57

2.4

Total

818

100.0

1582

100.0

2400

100.0

The frequency of pathological diagnoses

Pathological diagnoses were classified by pathogenesis (Table 3) and histopathology (Table 4). In the classification of pathogenesis, IgAN was diagnosed most frequently, followed by primary glomerular disease (except IgAN) and renal grafts both in 2007 and 2008 (Table 3). In the present cohort, except for renal grafts, the frequency of IgAN was 32.9%, followed by primary glomerular disease (except IgAN) (26.3%) and diabetic nephropathy (5.9%) in 2007 (Table 3). A slightly lower frequency of IgAN was present (30.2%), but similar frequencies of primary glomerular disease (except IgAN) (26.3%) and diabetic nephropathy (5.1%) were observed in 2008 (Table 3).
Table 3

Frequency of pathological diagnoses as classified by pathogenesis

Classification

2007

2008

Total

n

%

n

%

n

%

IgA nephropathy

239

29.2

424

26.8

663

27.6

Primary glomerular disease (except IgA nephropathy)

191

23.3

369

23.3

560

23.3

Renal graft

93

11.3

179

11.3

272

11.3

Diabetic nephropathy

43

5.2

71

4.5

114

4.8

Hypertensive nephrosclerosis

31

3.7

61

3.9

92

3.8

Lupus nephritis

29

3.5

59

3.7

88

3.7

MPO-ANCA-positive nephritis

25

3.0

58

3.7

83

3.5

Purpura nephritis

18

2.2

39

2.5

57

2.4

Amyloid nephropathy

12

1.4

22

1.4

34

1.4

Infection-related nephropathy

16

1.9

16

1.0

32

1.3

Thin basement membrane disease

11

1.3

5

0.3

16

0.7

Alport syndrome

1

0.1

9

0.6

10

0.4

PR3-ANCA-positive nephritis

1

0.1

7

0.4

8

0.3

Thrombotic microangiopathy

3

0.3

2

0.1

5

0.2

Anti-glomerular basement membrane antibody-type nephritis

0

0.0

4

0.3

4

0.2

Others

105

12.8

257

16.2

362

15.1

Total

818

100.0

1582

100.0

2400

100.0

Table 4

Frequency of pathological diagnoses as classified by histopathology

Classification

2007

2008

Total

n

%

n

%

n

%

Mesangial proliferative glomerulonephritis

326

39.8

607

38.4

933

38.9

Renal graft

90

11.0

171

10.8

261

10.9

Membranous nephropathy

74

9.0

128

8.1

202

8.4

Minor glomerular abnormalities

52

6.3

143

9.0

195

8.1

Crescentic and necrotizing glomerulonephritis

32

3.9

87

5.5

119

5.0

Nephrosclerosis

38

4.6

77

4.9

115

4.8

Focal segmental glomerulosclerosis

32

3.9

65

4.1

97

4.0

Membranoproliferative glomerulonephritis (type I and III)

20

2.4

32

2.0

52

2.2

Chronic interstitial nephritis

24

2.9

21

1.3

45

1.9

Endocapillary proliferative glomerulonephritis

18

2.2

27

1.7

45

1.9

Sclerosing glomerulonephritis

10

1.2

33

2.1

43

1.8

Acute interstitial nephritis

7

0.9

18

1.1

25

1.0

Acute tubular necrosis

5

0.6

6

0.4

11

0.5

Dense deposit disease

1

0.1

5

0.3

6

0.3

Others

89

10.8

162

10.2

251

10.5

Total

818

100.0

1582

100.0

2400

100.0

In the pathological diagnoses as classified by histopathology, mesangial proliferative glomerulonephritis was primarily observed in 2007 and 2008 (Table 4). In the present cohort, except for renal grafts, the frequency of mesangial proliferative glomerulonephritis was the highest followed by MN, minor glomerular abnormalities, nephrosclerosis, and crescentic and necrotizing glomerulonephritis in 2007 (Table 4). In 2008, mesangial proliferative glomerulonephritis was the most frequently diagnosed, with minor glomerular abnormalities being the second, and MN being the third (Table 4).

Primary glomerular disease (except IgAN) and nephrotic syndrome

In the cohort of primary glomerular disease as classified by pathogenesis, MN was predominant, followed by mesangial proliferative glomerulonephritis, minor glomerular abnormalities, and FSGS in 2007 (Table 5). In 2008, MN was still the most frequently diagnosed, present at the same frequency as minor glomerular abnormalities (Table 5).
Table 5

Frequency of pathological diagnoses as classified by histopathology in primary glomerular disease (except IgA nephropathy)

Classification

2007

2008

Total

n

%

n

%

n

%

Membranous nephropathy

60

31.4

95

25.7

155

27.7

Minor glomerular abnormalities

33

17.3

95

25.7

128

22.9

Mesangial proliferative glomerulonephritis

45

23.6

82

22.2

127

22.7

Focal segmental glomerulosclerosis

24

12.6

53

14.4

77

13.8

Membranoproliferative glomerulonephritis (type I and III)

13

6.8

19

5.1

32

5.7

Crescentic and necrotizing glomerulonephritis

5

2.6

6

1.6

11

2.0

Endocapillary proliferative glomerulonephritis

1

0.5

6

1.6

7

1.3

Nephrosclerosis

2

1.0

4

1.1

6

1.1

Dense deposit disease

1

0.5

3

0.8

4

0.7

Sclerosing glomerulonephritis

2

1.0

1

0.3

3

0.5

Others

5

2.6

5

1.4

10

1.8

Total

191

100.0

369

100.0

560

100.0

In nephrotic syndrome as classified by clinical diagnosis, primary glomerular disease (except IgAN) was predominant, followed by diabetic nephropathy, amyloid nephropathy, IgAN, and lupus nephritis in 2007 (Table 6). A similar ordering of the disease frequencies was noted in 2008 (Table 6). Among the primary glomerular diseases (except IgAN) in nephrotic syndrome, MN was dominant followed by minor glomerular abnormalities, such as minimal change nephrotic syndrome (MCNS), FSGS, and MPGN (type I and III) in 2007 (Table 7). In 2008, the frequency of minor glomerular abnormalities was predominant, followed by MN (Table 7).
Table 6

Frequency of pathological diagnoses as classified by pathogenesis in nephrotic syndrome

Classification

2007

2008

Total

n

%

n

%

n

%

Primary glomerular disease (except IgA nephropathy)

91

65.9

179

69.1

270

68.0

Diabetic nephropathy

15

10.9

15

5.8

30

7.6

Amyloid nephropathy

9

6.5

13

5.0

22

5.5

IgA nephropathy

8

5.8

9

3.5

17

4.3

Lupus nephritis

4

2.9

8

3.1

12

3.0

Purpura nephritis

1

0.7

4

1.5

5

1.3

Infection-related nephropathy

3

2.2

1

0.4

4

1.0

Thrombotic microangiopathy

1

0.7

0

0.0

1

0.3

MPO-ANCA-positive nephritis

0

0.0

1

0.4

1

0.3

Hypertensive nephrosclerosis

0

0.0

1

0.4

1

0.3

Others

6

4.3

28

10.8

34

8.6

Total

138

100.0

259

100.0

397

100.0

Table 7

Frequency of pathological diagnoses as classified by histopathology in primary glomerular disease (except IgA nephropathy) in nephrotic syndrome

Classification

2007

2008

Total

n

%

n

%

n

%

Minor glomerular abnormalities

29

31.9

79

44.1

108

40.0

Membranous nephropathy

40

44.0

56

31.3

96

35.6

Focal segmental glomerulosclerosis

10

11.0

25

14.0

35

13.0

Membranoproliferative glomerulonephritis (type I and III)

7

7.7

13

7.3

20

7.4

Mesangial proliferative glomerulonephritis

1

1.1

4

2.2

5

1.9

Crescentic and necrotizing glomerulonephritis

2

2.2

1

0.6

3

1.1

Endocapillary proliferative glomerulonephritis

1

1.1

0

0.0

1

0.4

Others

1

1.1

1

0.6

2

0.7

Total

91

100.0

179

100.0

270

100.0

Clinical diagnosis of MN, minor glomerular abnormalities, and FSGS

Subanalyses of subjects with a clinical diagnosis of MN, minor glomerular abnormalities, and FSGS were performed since these were the most common forms of primary glomerular diseases (except IgAN) (Tables 8, 9, 10). Nephrotic syndrome was the most common clinical diagnosis in MN and minor glomerular abnormalities (Tables 8, 9), whereas chronic nephritic syndrome was the most common in FSGS (Table 10). In the pathogenesis of minor glomerular abnormalities (total 195 cases), primary glomerular diseases (except IgAN) comprised 65.6% (128 cases), followed by others 13.8% (27 cases), IgAN 8.2% (16 cases) and thin basement membrane disease 5.1% (10 cases). In the pathogenesis of FSGS (total 97 cases), primary glomerular diseases (except IgAN) comprised 79.4% (77 cases), followed by others 11.3% (11 cases) and hypertensive nephrosclerosis 4.1% (4 cases).
Table 8

Frequency of clinical diagnoses in membranous nephropathy

Classification

2007

2008

Total

n

%

n

%

n

%

Nephrotic syndrome

44

59.5

66

51.6

110

54.5

Chronic nephritic syndrome

20

27.0

47

36.7

67

33.2

Renal disorder with collagen disease or vasculitis

7

9.5

9

7.0

16

7.9

Renal disorder with metabolic syndrome

1

1.4

1

0.8

2

1.0

Recurrent or persistent hematuria

1

1.4

0

0.0

1

0.5

Renal transplantation

0

0.0

1

0.8

1

0.5

Rapidly progressive nephritic syndrome

0

0.0

1

0.8

1

0.5

Acute nephritic syndrome

0

0.0

1

0.8

1

0.5

Drug-induced nephropathy

0

0.0

1

0.8

1

0.5

Others

1

1.4

1

0.8

2

1.0

Total

74

100.0

128

100.0

202

100.0

Table 9

Frequency of clinical diagnoses in minor glomerular abnormalities

Classification

2007

2008

Total

n

%

n

%

n

%

Nephrotic syndrome

29

55.8

82

57.3

111

56.9

Chronic nephritic syndrome

9

17.3

43

30.0

52

26.7

Recurrent or persistent hematuria

6

11.5

10

7.0

16

8.2

Renal disorder with collagen disease or vasculitis

1

1.9

5

3.5

6

3.1

Rapidly progressive nephritic syndrome

1

1.9

0

0.0

1

0.5

Renal disorder with metabolic syndrome

1

1.9

0

0.0

1

0.5

Acute nephritic syndrome

1

1.9

0

0.0

1

0.5

Drug-induced nephropathy

1

1.9

0

0.0

1

0.5

Inherited renal disease

0

0.0

1

0.7

1

0.5

Others

3

5.8

2

1.4

5

2.6

Total

52

100.0

143

100.0

195

100.0

Table 10

Frequency of clinical diagnoses in focal segmental glomerulosclerosis

Classification

2007

2008

Total

n

%

n

%

n

%

Chronic nephritic syndrome

18

56.3

32

49.2

50

51.5

Nephrotic syndrome

10

31.3

26

40.0

36

37.1

Inherited renal disease

2

6.3

0

0.0

2

2.1

Renal disorder with collagen disease or vasculitis

1

3.1

1

1.5

2

2.1

Rapidly progressive nephritic syndrome

1

3.1

1

1.5

2

2.1

Renal transplantation

0

0.0

1

1.5

1

1.0

Recurrent or persistent hematuria

0

0.0

1

1.5

1

1.0

Renal disorder with metabolic syndrome

0

0.0

1

1.5

1

1.0

Others

0

0.0

2

3.1

2

2.1

Total

32

100.0

65

100.0

97

100.0

Subanalysis of IgAN

The profile, classification of clinical diagnosis, and the pathological diagnosis of IgAN, the most frequent glomerulonephritis on the J-RBR, were further analyzed (Tables 11, 12, 13). The percentage of IgAN detected in total biopsies and native kidneys was 27.5 and 31.0% in 2007 and 2008, respectively. The average age was the fourth decade in both genders. There was no difference in the proportion based on gender (Table 11). The majority of the clinical and pathological diagnoses were chronic nephritic syndrome (Table 12) and mesangial proliferative glomerulonephritis (Table 13), respectively.
Table 11

Profile of IgA nephropathy

IgA nephropathy

2007

2008

Total

Total native kidney biopsies (n)

239

421

660

 Average age (y)

36.5 ± 19.0

36.4 ± 18.2

36.4 ± 18.5

Male (n)

112 (46.9%)a

219 (52.0%)a

331 (50.2%)a

 Average age (y)

37.1 ± 18.9b

37.2 ± 19.3b

37.2 ± 19.1b

Female (n)

127 (53.1%)

202 (48.0%)

329 (49.8%)

 Average age (y)

36.1 ± 19.2

35.4 ± 17.0

35.7 ± 17.8

aRatio indicates percentage of each gender in each biopsy category

bNot significant as compared to another gender

Table 12

Frequency of classification of clinical diagnoses in IgA nephropathy

Clinical diagnosis

2007

2008

Total

n

%

n

%

n

%

Chronic nephritic syndrome

197

82.4

387

91.9

584

88.5

Recurrent or persistent hematuria

23

9.6

17

4.0

40

6.1

Nephrotic syndrome

8

3.3

9

2.1

17

2.6

Rapidly progressive nephritic syndrome

8

3.3

1

0.2

9

1.4

Acute nephritic syndrome

2

0.8

4

0.9

6

0.9

Hypertensive nephropathy

0

0.0

2

0.5

2

0.3

Renal disorder with metabolic disease

1

0.4

0

0.0

1

0.2

Acute renal failure

0

0.0

1

0.2

1

0.2

Total

239

100.0

421

100.0

660

100.0

Table 13

Frequency of pathological diagnoses as classified by histopathology

Pathological diagnosis by histopathology

2007

2008

Total

n

%

n

%

n

%

Mesangial proliferative glomerulonephritis

228

95.4

398

94.5

626

94.8

Minor glomerular abnormalities

0

0.0

16

3.8

16

2.4

Crescentic and necrotizing glomerulonephritis

2

0.8

3

0.7

5

0.8

Sclerosing glomerulonephritis

3

1.3

0

0.0

3

0.5

Nephrosclerosis

1

0.4

1

0.2

2

0.3

Membranous nephropathy

1

0.4

1

0.2

2

0.3

Membranoproliferative glomerulonephritis (type I and III)

1

0.4

0

0.0

1

0.2

Others

3

1.3

2

0.5

5

0.8

Total

239

100.0

421

100.0

660

100.0

Other diseases

Rare diseases such as Alport syndrome, Fabry disease, lipoprotein glomerulopathy, and dense deposit disease (one case each) were registered in 2007, and one subject was diagnosed with POEMS syndrome in 2008.

Discussion

The J-RBR obtained data from 818 and 1582 patients with kidney disease and renal transplantation who submitted renal biopsies in 2007 and 2008, respectively. The main objectives of the registry were, based on the histopathological findings, to establish the frequency of glomerulopathies, tubulointerstitial diseases, renal vascular disorders, and renal grafts in renal biopsies in Japan. Data for all patients with histopathological evidence of renal disease at the participating centers were collected on standard forms and registered on the J-RBR program in the UMIN-INDICE. Chronic nephritic syndrome was the most frequent clinical diagnosis in both years of the registry. IgAN was the most frequently diagnosed disease in renal biopsies in 2007 and 2008, consistent with previous reports [8]. In patients with nephrotic syndrome, primary glomerular diseases (except IgAN) were predominant in both years.

Regarding the classification of clinical diagnosis in native kidney biopsies, more than half were diagnosed with chronic nephritic syndrome, which was usually accompanied by urinary abnormalities, as shown in Table 2. The frequency of clinical diagnosis may reflect the prevalence of renal biopsy in Japan. Indications of renal biopsy in Japan included urinary abnormalities such as mild-to-moderate proteinuria with or without hematuria, massive proteinuria such as nephrotic syndrome, rapidly progressive glomerulonephritis, and renal allografts (a protocol or episode biopsy). Solitary hematuria may be indicated after urological examinations. In Japan, all students in primary and junior high schools routinely undergo an annual urinalysis by the dip-stick test as one of the national health programs. Therafter students in high schools and universities and employees of companies submit to a urinalysis as part of a nationwide screening program. This social system promotes the early referral to nephrologists and may thus influence the frequency of chronic nephritic syndrome according to the clinical diagnoses of the J-RBR.

In the present study, IgAN was the most frequently diagnosed by pathological findings, which is consistent with a previous report [8]. The frequency of IgAN was 32.9% in 2007 and 30.2% in 2008 in native kidneys of patients registered on the J-RBR, which was less than that in the previous nationwide survey [8]. IgAN is the most common biopsy-proven renal disease among primary glomerulopathies in Asia as described in reports from Korea [12] and China [13]. In the United States, IgAN is the most common primary glomerulopathy in young adult Caucasians and the most common cause of end-stage renal disease, while it was found to be rare in African Americans in whom FSGS remained more common [14]. In Australia, IgAN, FSGS, lupus nephritis, and vasculitis are the most common renal diseases in adults with a male predominance, excepting lupus nephritis [6]. In Europe, IgAN is the most frequent primary glomerulonephritis in several countries [2, 4, 5, 15], while MN is the most frequent in Macedonia [16], MPGN in Romania [17], and non-IgA mesangial proliferative glomerulonephritis in Serbia [18]. FSGS is the most frequent renal disease in a recent report from Brazil [19]. Because there is a different policy of renal biopsy practice in each country, it may not be easy to compare the different databases across countries. Instead, the changing frequency patterns of renal disease in the same country over a certain time period are useful to treat disease and reduce chronic kidney disease burden [20].

The frequency of nephrotic syndrome was 19.0% in 2007 and 18.5% in 2008 for patients registered on the J-RBR. Primary renal diseases were present in approximately two-thirds of all patients with nephrotic syndrome. MN was the most common primary nephrotic syndrome in 2007 (44.0%) and MCNS was the most common in 2008 (44.1%). The reason for this difference may depend on the cohort of registered biopsies in both years, since the number of patients registered was not as large as other registries [2, 4, 13, 19].

For the registry of patients with end-stage renal disease in Japan, there has been a nationwide and yearly statistical survey of chronic dialysis patients since 1968, conducted by the Japanese Society for Dialysis Therapy in Japan [21]. The combined data of the J-RBR with this dialysis registry will allow us to evaluate the long-term outcome of patients with various renal diseases in the near future. Similarly, the combined renal transplant registry data allows the evaluation of patient outcome. A sizeable frequency of renal grafts was registered on the J-RBR. Consequently, the future analysis of renal grafts, including the frequency of the protocol and episode biopsies and the precise histological diagnosis, will be necessary.

There is no overall registry of renal biopsies in Japan at the moment. It is noteworthy that the J-RBR is web-based, and a prospective registry system that can easily increase the number of participating centers and enlarge the number of patients enroled in the future. We cannot conclude that the present sample of patients on the J-RBR in 2007 and in 2008 is actually representative of the nationwide frequency of glomerular, tubulointerstitial, or renal vascular diseases or renal grafts in Japan. However, in the near future, investigation of a larger cohort or a population-based analysis of the rate of each renal disease may reveal the actual frequency of the disease and the distribution of age ranges by utilizing the J-RBR system.

Notes

Acknowledgments

The authors greatly acknowledge the help and assistance of many colleagues in centers and affiliated hospitals with collecting the data. We also sincerely thank Ms. Mayumi Irie in the UNIN-INDICE for establishing and supporting the registration system of J-RBR. This study was supported by the committee grant from the Japanese Society of Nephrology and by a grant-in-aid from the Research Group on Progressive Renal Disease from the Ministry of Health, Labor and Welfare, Japan.

Supplementary material

10157_2011_430_MOESM1_ESM.doc (38 kb)
Supplementary Table (DOC 38 kb)

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Copyright information

© Japanese Society of Nephrology 2011

Authors and Affiliations

  • Hitoshi Sugiyama
    • 1
  • Hitoshi Yokoyama
    • 2
    Email author
  • Hiroshi Sato
    • 3
  • Takao Saito
    • 4
  • Yukimasa Kohda
    • 5
  • Shinichi Nishi
    • 6
    • 26
  • Kazuhiko Tsuruya
    • 7
  • Hideyasu Kiyomoto
    • 8
  • Hiroyuki Iida
    • 9
  • Tamaki Sasaki
    • 10
  • Makoto Higuchi
    • 11
  • Motoshi Hattori
    • 12
  • Kazumasa Oka
    • 13
    • 27
  • Shoji Kagami
    • 14
  • Michio Nagata
    • 15
  • Tetsuya Kawamura
    • 16
  • Masataka Honda
    • 17
    • 28
  • Yuichiro Fukasawa
    • 18
  • Atsushi Fukatsu
    • 19
  • Kunio Morozumi
    • 20
  • Norishige Yoshikawa
    • 21
  • Yukio Yuzawa
    • 22
    • 29
  • Seiichi Matsuo
    • 22
  • Yutaka Kiyohara
    • 23
  • Kensuke Joh
    • 24
    • 30
  • Takashi Taguchi
    • 25
    Email author
  • Hirofumi Makino
    • 1
  • Committee for Standardization of Renal Pathological Diagnosis and Working Group for Renal Biopsy Database, Japanese Society of Nephrology, Tokyo, Japan
  1. 1.Department of Medicine and Clinical ScienceOkayama University Graduate School of Medicine, Dentistry, and Pharmaceutical SciencesOkayamaJapan
  2. 2.Division of NephrologyKanazawa Medical University School of MedicineIshikawaJapan
  3. 3.Division of NephrologyTohoku University Graduate School of MedicineSendaiJapan
  4. 4.Division of Nephrology and Rheumatology, Department of Internal MedicineFukuoka University School of MedicineFukuokaJapan
  5. 5.Department of Nephrology, Faculty of Life SciencesKumamoto UniversityKumamotoJapan
  6. 6.Blood Purification CenterNiigata University Medical and Dental HospitalNiigataJapan
  7. 7.Department of Integrated Therapy for Chronic Kidney Disease, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  8. 8.Division of Nephrology and Dialysis, Department of Cardiorenal and Cerebrovascular Medicine, Faculty of MedicineKagawa UniversityTakamatsuJapan
  9. 9.Department of Internal MedicineToyama Prefectural Central HospitalToyamaJapan
  10. 10.Division of Nephrology and Hypertension, Department of Internal MedicineKawasaki Medical SchoolKurashikiJapan
  11. 11.Division of Nephrology, Department of Internal MedicineShinshu University School of MedicineMatsumotoJapan
  12. 12.Department of Pediatric NephrologyTokyo Women’s Medical University, School of MedicineTokyoJapan
  13. 13.Department of PathologyOsaka Kaisei HospitalOsakaJapan
  14. 14.Department of Pediatrics, Institute of Health BioscienceThe University of Tokushima Graduate SchoolTokushimaJapan
  15. 15.Molecular Pathology, Biomolecular and Integrated Medical Sciences, Graduate School of Comprehensive Human SciencesUniversity of TsukubaTsukubaJapan
  16. 16.Division of Kidney and Hypertension, Department of MedicineJikei University School of MedicineTokyoJapan
  17. 17.Department of Pediatric NephrologyTokyo Metropolitan Kiyose Children’s HospitalTokyoJapan
  18. 18.Department of PathologyKKR Sapporo Medical CenterSapporoJapan
  19. 19.Department of NephrologyKyoto University Graduate School of MedicineKyotoJapan
  20. 20.Kidney CenterJapanese Red Cross Nagoya Daini HospitalNagoyaJapan
  21. 21.Department of PediatricsWakayama Medical University, School of MedicineWakayamaJapan
  22. 22.Department of NephrologyNagoya University Graduate School of MedicineNagoyaJapan
  23. 23.Department of Environmental Medicine, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  24. 24.Division of Renal Pathology, Clinical Research CenterChiba-East National HospitalChibaJapan
  25. 25.Department of PathologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
  26. 26.Division of Nephrology and Kidney CenterKobe University Graduate School of MedicineKobeJapan
  27. 27.Department of PathologyHyogo Prefectural Nishinomiya HospitalNishinomiyaJapan
  28. 28.Tokyo Metropolitan Children’s Medical CenterTokyoJapan
  29. 29.Department of NephrologyFujita Health UniversityToyoakeJapan
  30. 30.Division of PathologySendai Shakai Hoken HospitalSendaiJapan

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