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Clinical and Experimental Nephrology

, Volume 17, Issue 2, pp 155–173 | Cite as

Japan Renal Biopsy Registry and Japan Kidney Disease Registry: Committee Report for 2009 and 2010

  • 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
  • Tetsuya Kawamura
  • Tetsuro Takeda
  • Hiroshi Hataya
  • Yuichiro Fukasawa
  • Atsushi Fukatsu
  • Kunio Morozumi
  • Norishige Yoshikawa
  • Akira Shimizu
  • Hiroshi Kitamura
  • Yukio Yuzawa
  • Seiichi Matsuo
  • Yutaka Kiyohara
  • Kensuke Joh
  • Michio Nagata
  • Takashi TaguchiEmail author
  • Hirofumi Makino
  • Committee for Standardization of Renal Pathological Diagnosis and Committee for Kidney Disease Registry, Japanese Society of Nephrology, Japan
Special Report

Abstract

The Japan Renal Biopsy Registry (J-RBR) was started in 2007 and the Japan Kidney Disease Registry (J-KDR) was then started in 2009 by the Committee for Standardization of Renal Pathological Diagnosis and the Committee for the Kidney Disease Registry of the Japanese Society of Nephrology. The purpose of this report is to describe and summarize the registered data from 2009 and 2010. For the J-KDR, data were collected from 4,016 cases, including 3,336 (83.1 %) by the J-RBR and 680 (16.9 %) other cases from 59 centers in 2009, and from 4,681 cases including 4,106 J-RBR cases (87.7 %) and 575 other cases (12.3 %) from 94 centers in 2010, including the affiliate hospitals. In the J-RBR, 3,165 native kidneys (94.9 %) and 171 renal grafts (5.1 %) and 3,869 native kidneys (94.2 %) and 237 renal grafts (5.8 %) were registered in 2009 and 2010, respectively. Patients younger than 20 years of age comprised 12.1 % of the registered cases, and those 65 years and over comprised 24.5 % of the cases with native kidneys in 2009 and 2010. The most common clinical diagnosis was chronic nephritic syndrome (55.4 % and 50.0 % in 2009 and 2010, respectively), followed by nephrotic syndrome (22.4 % and 27.0 %); the most frequent pathological diagnosis as classified by the pathogenesis was IgA nephropathy (31.6 % and 30.4 %), followed by primary glomerular diseases (except IgA nephropathy) (27.2 % and 28.1 %). Among the primary glomerular diseases (except IgA nephropathy) in the patients with nephrotic syndrome, membranous nephropathy was the most common histopathology in 2009 (40.3 %) and minor glomerular abnormalities (50.0 %) were the most common in 2010 in native kidneys in the J-RBR. Five new secondary and longitudinal research studies by the J-KDR were started in 2009 and one was started in 2010.

Keywords

Native kidney biopsy Primary glomerulonephritis IgA nephropathy Membranous nephropathy Renal grafts National registry 

Introduction

The Japanese Society of Nephrology (JSN) established the Japan Renal Biopsy Registry (J-RBR) in 2007, and it conducted analyses for 2007 and 2008 [1]. In 2009, the JSN started the Japan Kidney Disease Registry (J-KDR) to record clinically-diagnosed cases in addition to the J-RBR. Participation in the J-KDR, including the J-RBR, was requested from appointed clinical training hospitals of the JSN and the Japanese Society for Dialysis Therapy in an attempt to extend the registry nationwide. In this report, the detailed data of the J-RBR and the frequencies of the different clinical diagnoses in the J-KDR registered from January to December of 2009 and 2010 are summarized.

Subjects and methods

Registry system and patients

This report includes the data from patients included in the J-RBR and J-KDR (J-RBR/J-KDR), registered prospectively from January 2009 to December 2010. The patients’ data, including age, gender, laboratory data, and the clinical and pathological diagnoses, were recorded at each institution and registered on the web page of the J-RBR/J-KDR utilizing the Internet Data and Information Center for Medical Research (INDICE) system of the University Hospital Medical Information Network (UMIN), as described previously [1]. The ethics committee of the JSN and that of Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences comprehensively approved the study, and a local committee of participating centers and their affiliate hospitals individually approved the study. Written informed consent was obtained from the patients at the time of biopsy or at the time they were registered to participate in the study. The J-RBR/J-KDR is registered in the Clinical Trial Registry of UMIN (Registered Number UMIN000000618).

Clinical or renal histopathological diagnosis and laboratory data

Three classifications, including the clinical diagnosis, histological diagnosis based on the pathogenesis, and histological diagnosis based on a histopathological examination, were made for each case included in the J-RBR, as described previously [1]. Of these classifications, the clinical diagnosis alone was selected for the J-KDR. The definition of each diagnosis was based on the clinical syndromes and renal histopathology, as described previously [2]. IgA nephropathy (IgAN) (Berger disease) was separated from primary glomerular diseases on the basis of basic glomerular alterations in the classification of glomerular diseases by the World Health Organization [2]. In 2010, hemolytic uremic syndrome and thrombotic thrombocytopenic purpura (HUS/TTP), congenital anomalies of the kidney and urinary tract (CAKUT) and polycystic kidney disease (PKD) were added to the classification of the clinical diagnosis on the case record (Table S1). The clinical data, including the results of the urinalysis, daily proteinuria, serum creatinine concentrations, total protein, albumin, and the total cholesterol values, were always recorded, while the systolic and diastolic blood pressure, prescription use of anti-hypertensive agents, hemoglobin A1c, and presence of diabetes mellitus were optionally recorded. The estimated glomerular filtration rate was calculated as described previously [3]. The frequency of the diseases are here described in general, but the clinical data were also analyzed separately for cases of IgAN, which is the most common renal disease in Japan [1, 4, 5].

Statistical analyses

Data are expressed as the mean ± SD for continuous parametric data, medians and interquartile ranges for continuous non-parametric data, and frequencies for categorical data. The statistical analyses were performed using the JMP software program, version 8 (SAS Institute Inc., Cary, NC, USA).

Results

Baseline characteristics of the J-RBR/J-KDR participants in 2009 and 2010

The numbers of participating facilities and registered renal biopsies or cases without renal biopsies in the registry in 2009 and 2010 are shown in Table 1. The J-KDR was started in 2009 and the number of participating facilities increased by 34 compared to 2008, reaching a total of 57 facilities in the J-RBR and 59 facilities in the J-KDR. The number of total renal biopsies increased to 3,336 in 2009, which was 1,754 more biopsies than in the previous year [1], and in 2010 it further increased to 4,106 in the J-RBR. The number of other cases (not in the J-RBR), which corresponds to the cases without renal biopsies but diagnosed by clinical findings, was 680 and 575 in 2009 and 2010, respectively. The average age of this cohort was more than 10 years higher than that of the J-RBR in each year (Table 1).
Table 1

The number of participated renal centers and registered renal biopsies or other cases without renal biopsies in J-RBR/J-KDR 2009 and 2010

 

2009 J-KDR

2010 J-KDR

J-RBR

Other casesa

Total

J-RBR

Other casesa

Total

Renal centers (n)b

57c

59

83

94

Total biopsies or cases (n)

3,336d (83.1 %)

680 (16.9 %)

4,016 (100.0 %)

4,106 (87.7 %)

575 (12.3 %)

4,681 (100.0 %)

Average age (years)

46.7 ± 19.9

58.1 ± 17.8

48.7 ± 20.0

46.7 ± 20.6

56.8 ± 21.1

47.9 ± 20.9

Male (n)

1,787 (53.6 %)

418 (61.5 %)

2,205 (54.9 %)

2,183 (53.2 %)

335e (58.3 %)

2,518e (53.8 %)

Female (n)

1,549 (46.4 %)

262 (38.5 %)

1,811 (45.1 %)

1,923 (46.8 %)

238e (41.4 %)

2,161e (46.2 %)

J-RBR Japan Renal Biopsy Registry, J-KDR Japan Kidney Disease Registry

Note that J-RBR started in 2007 and J-KDR started in 2009

aOther cases include patients diagnosed by clinical findings without renal biopsies

bThe number represents principal institutions having affiliate hospitals. All of the participated institutions and hospitals in the J-RBR and J-KDR in 2009 and 2010 are shown in the “Appendix”. The number of renal centers in total is based on the registration of cases without renal biopsies but diagnosed by clinical findings in addition to that of data with renal biopsy in J-RBR

cIncrease of 34 when compared to the number in J-RBR 2008

dIncrease of 1,754 when compared to the number in J-RBR 2008

eNo registered data for gender in 2 cases

The number of native kidney biopsies increased; however, that of renal graft biopsies registered in 2009 slightly decreased compared to 2008 (Table 2). The distribution of age ranges showed a peak distribution in the seventh decade in both genders for native kidneys (Table 3). Patients younger than 20 years of age comprised 12.1 % and 10.3 % of the cases, and those 65 years of age and over comprised 24.5 % and 4.7 % of the native kidney and renal grafts, respectively, during the 2-year period (2009 and 2010). In the patients who underwent renal grafts, both the average age and the peak distribution of age ranges were younger than those of patients who underwent native kidney biopsies (Tables 2, 3).
Table 2

The number of registered renal biopsies in J-RBR 2009 and 2010

Years

2009

2010

Total

Native kidneys, n (%)

3,165a (94.9)

3,869 (94.2)

7,034 (94.5)

 Average age (years)

47.0 ± 20.1

47.1 ± 20.8

47.1 ± 20.5

 Median age (years)

50 (30–64)

49 (31–65)

49 (30–64)

 Male, n (%)

1,671 (52.8)

2,035 (52.6)

3,706 (52.7)

 Female, n (%)

1,494 (47.2)

1,834 (47.4)

3,328 (47.3)

Renal grafts, n (%)

171b (5.1)

237 (5.8)

408 (5.5)

 Average age (years)

40.9 ± 15.0

41.3 ± 15.4

41.1 ± 15.2

 Median age (years)

43 (31–52)

41 (33–54)

42 (32–53)

 Male, n (%)

116 (67.8)

148 (62.4)

264 (64.7)

 Female, n (%)

55 (32.2)

89 (37.6)

144 (35.3)

aIncrease of 1,765 when compared to the number in J-RBR 2008

bDecrease of 11 when compared to the number in J-RBR 2008

Table 3

Distribution of age ranges and gender in J-RBR 2009 and 2010

 

2009

2010

Total biopsies (n = 3,336)

Native kidneys (n = 3,165)

Renal grafts (n = 171)

Total biopsies (n = 4,106)

Native kidneys (n = 3,869)

Renal grafts (n = 237)

Age (years)

Total

Male

Female

Total

Male

Female

Total

Male

Female

Total

Male

Female

Total

Male

Female

Total

Male

Female

0–9

60

33

27

57

32

25

3

1

2

121

94

27

136

87

49

7

7

0

10–19

318

169

149

304

160

144

14

9

5

352

203

149

354

193

161

18

10

8

20–29

413

194

219

392

180

212

21

14

7

406

187

219

429

167

262

22

20

2

30–39

476

221

255

438

193

245

38

28

10

533

278

255

549

248

301

62

30

32

40–49

434

222

212

391

197

194

43

25

18

489

277

212

489

251

238

50

26

24

50–59

545

317

228

509

291

218

36

26

10

575

347

228

541

311

230

49

36

13

60–69

645

382

263

631

371

260

14

11

3

733

470

263

756

452

304

28

18

10

70–79

372

213

159

370

211

159

2

2

0

437

278

159

515

277

238

1

1

0

80+

73

36

37

73

36

37

0

0

0

86

49

37

100

49

51

0

0

0

Total

3,336

1,787

1,549

3,165

1,671

1,494

171

116

55

3,732

2,183

1,549

3,869

2,035

1,834

237

148

89

Under 20 (%)

11.3

11.3

11.4

11.4

11.5

11.3

9.9

8.6

12.7

12.5

13.6

11.4

12.7

13.8

11.5

10.5

11.5

9.0

65 and over (%)

22.4

23.9

20.1

23.4

25.3

21.3

4.7

4.3

5.5

24.2

25.4

20.7

25.4

26.9

23.7

4.6

5.4

3.4

Age (years)

Total

         

Total biopsies (n = 7,442)

Native kidneys (n = 7,034)

Renal grafts (n = 408)

         

Total

Male

Female

Total

Male

Female

Total

Male

Female

         

0–9

203

127

76

193

119

74

10

8

2

         

10–19

690

372

318

658

353

305

32

19

13

         

20–29

864

381

483

821

347

474

43

34

9

         

30–39

1,087

499

588

987

441

546

100

58

42

         

40–49

973

499

474

880

448

432

93

51

42

         

50–59

1,135

664

471

1,050

602

448

85

62

23

         

60–69

1,429

852

577

1,387

823

564

42

29

13

         

70–79

888

491

397

885

488

397

3

3

0

         

80+

173

85

88

173

85

88

0

0

0

         

Total

7,442

3,970

3,472

7,034

3,706

3,328

408

264

144

         

Under 20 (%)

12.0

12.6

11.3

12.1

12.7

11.4

10.3

10.2

10.4

         

65 and over (%)

23.4

24.7

21.8

24.5

26.1

22.6

4.7

4.9

4.2

         

The frequency of clinical diagnoses in the J-RBR

Three classifications, the clinical diagnosis, histological diagnosis based on the pathogenesis, and the histological diagnosis based on a histopathological examination, were included in the J-RBR database, while the clinical diagnosis alone was registered for the other cases. In the J-RBR, a clinical diagnosis of chronic nephritic syndrome was the most common, followed by nephrotic syndrome, in both total biopsies and native kidneys in 2009 and 2010, which was similar to the findings in 2007 and 2008 (Table 4) [1]. In native kidneys, more than half of the cases that were registered had chronic nephritic syndrome. The age distribution according to the classification of clinical diagnoses in native kidneys in the J-RBR in 2009 and 2010 was analyzed, and cases with rapidly progressive nephritic syndrome exhibited the highest mean age while cases with inherited renal diseases showed the youngest mean age (Table 5).
Table 4

The frequency of classification of clinical diagnoses in J-RBR 2009 and 2010

Classification

2009

2010

Total

Total biopsies (n = 3,336)

Native kidneys (n = 3,165)

Total biopsies (n = 4,106)

Native kidneys (n = 3,869)

Total biopsies (n = 7,442)

Native kidneys (n = 7,034)

n

%

%a

n

%

%a

n

%

%a

Chronic nephritic syndrome

1,759

52.7

55.4

1,944

47.3

50.0

3,703

49.8

52.5

Nephrotic syndrome

711

21.3

22.4

1,044

25.4

27.0

1,755

23.6

24.9

Rapidly progressive nephritic syndrome

200

6.0

6.3

292

7.1

7.5

492

6.6

7.0

Renal transplantation

160

4.8

227

5.5

387

5.2

Renal disorder with collagen disease or vasculitis

116

3.5

3.7

144

3.5

3.7

260

3.5

3.7

Recurrent or persistent hematuria

97

2.9

3.0

111

2.7

2.9

208

2.8

2.9

Renal disorder with metabolic disease

63

1.9

2.0

61

1.5

1.6

124

1.7

1.8

Acute nephritic syndrome

54

1.6

1.6

58

1.4

1.5

112

1.5

1.6

Hypertensive nephropathy

39

1.2

1.2

54

1.3

1.4

93

1.3

1.3

Acute renal failure

36

1.1

1.1

35

0.9

0.9

71

1.0

1.0

Drug-induced nephropathy

13

0.4

0.4

26

0.6

0.6

39

0.5

0.5

Inherited renal disease

6

0.2

0.2

15

0.4

0.4

21

0.3

0.3

HUS/TTP

3

0.1

0.1

3

0.0

0.0

Others

82

2.5

2.6

92

2.2

2.4

174

2.4

2.5

Total

3,336

100.0

100.0

4,106

100.0

100.0

7,442

100.0

100.0

aPatients classified as either “Renal graft” or “Renal transplantation” in other categories were also excluded

Table 5

The age distribution of classification of clinical diagnoses in native kidneys in J-RBR 2009 and 2010

Classification

2009

2010

Total

Male

Female

Total

Male

Female

Total

Male

Female

Total

Chronic nephritic syndrome

44.4 ± 18.8

41.2 ± 17.8

42.8 ± 18.4

43.5 ± 19.3

41.0 ± 18.2

42.2 ± 18.8

43.9 ± 19.1

41.0 ± 18.0

42.5 ± 18.6

Nephrotic syndrome

52.6 ± 21.6

54.7 ± 21.1

53.5 ± 21.4

49.5 ± 23.4

50.9 ± 22.6

50.1 ± 23.0

50.8 ± 22.7

52.5 ± 22.0

51.5 ± 22.4

Rapidly progressive nephritic syndrome

64.5 ± 13.0

61.2 ± 17.4

63.0 ± 15.1

65.4 ± 11.5

65.3 ± 15.3

65.4 ± 13.3

65.1 ± 12.1

63.6 ± 16.3

64.4 ± 14.1

Renal disorder with collagen disease or vasculitis

48.0 ± 21.5

46.2 ± 20.1

46.7 ± 20.4

54.3 ± 19.5

46.3 ± 19.6

48.7 ± 19.9

51.6 ± 20.5

46.2 ± 19.8

47.8 ± 20.1

Recurrent or persistent hematuria

33.4 ± 17.4

33.8 ± 16.9

33.6 ± 17.0

49.5 ± 19.0

38.0 ± 17.1

42.6 ± 18.6

41.8 ± 19.9

36.1 ± 17.0

38.4 ± 18.4

Renal disorder with metabolic disease

56.9 ± 12.3

57.9 ± 8.9

57.2 ± 11.5

56.8 ± 14.8

54.8 ± 14.1

56.2 ± 14.5

56.9 ± 13.5

56.2 ± 11.9

56.7 ± 13.0

Acute nephritic syndrome

42.8 ± 19.2

36.0 ± 22.5

39.9 ± 20.7

49.6 ± 17.5

46.6 ± 21.1

48.1 ± 19.3

46.1 ± 18.5

42.0 ± 22.1

44.2 ± 20.3

Hypertensive nephropathy

56.2 ± 13.5

51.0 ± 15.3

55.2 ± 13.8

54.5 ± 15.9

54.7 ± 17.0

54.6 ± 16.0

55.3 ± 14.8

53.3 ± 16.1

54.8 ± 15.1

Acute renal failure

56.0 ± 19.3

56.4 ± 26.2

56.1 ± 21.2

55.2 ± 17.6

58.0 ± 20.6

56.0 ± 18.2

55.6 ± 18.3

57.1 ± 23.1

56.0 ± 19.7

Drug-induced nephropathy

53.6 ± 11.9

35.2 ± 21.6

45.1 ± 18.9

47.3 ± 20.0

60.4 ± 17.6

51.5 ± 19.9

49.1 ± 18.0

49.6 ± 22.7

49.3 ± 19.5

Inherited renal disease

25.0 ± 23.8

40.7 ± 24.1

32.8 ± 23.1

15.0 ± 17.1

24.3 ± 25.3

19.3 ± 21.1

17.7 ± 18.5

29.2 ± 24.9

23.2 ± 22.0

HUS/TTP

10, 69

49

42.6 ± 30.0

10, 69

49

42.6 ± 30.0

Others

50.6 ± 18.2

48.4 ± 19.5

49.6 ± 18.7

48.6 ± 20.9

53.3 ± 18.1

50.5 ± 19.8

49.4 ± 19.6

50.9 ± 18.9

50.0 ± 19.2

Total

48.4 ± 20.0

45.5 ± 20.0

47.0 ± 20.1

48.2 ± 21.0

46.0 ± 20.5

47.1 ± 20.8

48.3 ± 20.6

45.8 ± 20.3

47.1 ± 20.5

The frequency of pathological diagnoses in the J-RBR

The pathological diagnoses were classified based on the pathogenesis (Table 6) and histopathology (Table 7). In the classification of the pathogenesis, IgAN was diagnosed most frequently (31.6 %), followed by primary glomerular disease other than IgAN (27.2 %) in native kidneys in both 2009 and 2010 (Table 6). Similar frequencies of IgAN, primary glomerular disease other than IgAN and diabetic nephropathy were observed in the combined data for 2007 and 2008 [1]. In the pathological diagnosis classified based on the histopathology in native kidney biopsies, mesangial proliferative glomerulonephritis was the most frequently observed disease, representing 42.5 % and 35.8 % of the cases in 2009 and 2010 (Table 7).
Table 6

The frequency of pathological diagnoses as classified by pathogenesis in J-RBR 2009 and 2010

Classification

2009

2010

Total

Total biopsies (n = 3,336)

Native kidneys (n = 3,165)

Total biopsies (n = 4,106)

Native kidneys (n = 3,869)

Total biopsies (n = 7,442)

Native kidneys (n = 7,034)

n

%

%a

n

%

%a

n

%

%a

IgA nephropathy

1,003

30.1

31.6

1,177

28.7

30.4

2,180

29.3

31.0

Primary glomerular disease (except IgA nephropathy)

862

25.8

27.2

1,090

26.5

28.1

1,952

26.2

27.7

Diabetic nephropathy

184

5.5

5.8

192

4.7

5.0

376

5.1

5.3

Renal graft

161

4.8

235

5.7

396

5.3

Lupus nephritis

137

4.1

4.3

220

5.4

5.7

357

4.8

5.1

MPO-ANCA positive nephritis

129

3.9

4.1

191

4.7

4.9

320

4.3

4.5

Hypertensive nephrosclerosis

123

3.7

3.9

157

3.8

4.1

280

3.8

4.0

Purpura nephritis

64

1.9

2.0

108

2.6

2.8

172

2.3

2.4

Amyloid nephropathy

45

1.3

1.4

58

1.4

1.5

103

1.4

1.5

Infection-related nephropathy

27

0.8

0.9

31

0.8

0.8

58

0.8

0.8

Thin basement membrane disease

26

0.8

0.8

39

1.0

1.0

65

0.9

0.9

PR3-ANCA positive nephritis

13

0.4

0.4

11

0.3

0.3

24

0.3

0.3

Alport syndrome

10

0.3

0.3

16

0.4

0.4

26

0.3

0.4

Thrombotic microangiopathy

9

0.3

0.3

8

0.2

0.2

17

0.2

0.2

Anti-GBM antibody-type nephritis

8

0.2

0.3

16

0.4

0.4

24

0.3

0.3

Others

535

16.0

16.7

557

13.6

13.6

1,092

14.7

15.4

Total

3,336

100.0

100.0

4,106

100.0

100.0

7,442

100.0

100.0

MPO myeloperoxidase, ANCA anti-neutrophil cytoplasmic antibody, PR3 proteinase 3, GBM glomerular basement membrane

aPatients classified as either “Renal graft” or “Renal transplantation” in other categories were also excluded

Table 7

The frequency of pathological diagnoses as classified by histopathology in J-RBR 2009 and 2010

Classification

2009

2010

Total

Total biopsies (n = 3,336)

Native kidneys (n = 3,165)

Total biopsies (n = 4,106)

Native kidneys (n = 3,869)

Total biopsies (n = 7,442)

Native kidneys (n = 7,034)

n

%

%a

n

%

%a

n

%

%a

Mesangial proliferative glomerulonephritis

1,346

40.3

42.5

1,388

33.8

35.8

2,734

36.7

38.8

Membranous nephropathy

333

10.0

10.5

418

10.2

10.8

751

10.1

10.7

Minor glomerular abnormality

293

8.8

9.2

559

13.6

14.4

852

11.4

12.1

Crescentic and necrotizing glomerulonephritis

180

5.4

5.7

262

6.4

6.8

442

5.9

6.3

Focal segmental glomerulosclerosis

167

5.0

5.2

211

5.1

5.4

378

5.1

5.3

Nephrosclerosis

163

4.9

5.2

208

5.1

5.4

371

5.0

5.3

Renal graft

151

4.5

227

5.5

378

5.1

Membranoproliferative glomerulonephritis (types I and III)

85

2.5

2.7

97

2.4

2.5

182

2.4

2.6

Chronic interstitial nephritis

71

2.1

2.1

61

1.5

1.6

132

1.7

1.8

Sclerosing glomerulonephritis

63

1.9

2.0

44

1.1

1.1

107

1.4

1.5

Endocapillary proliferative glomerulonephritis

61

1.8

1.9

67

1.6

1.7

128

1.7

1.8

Acute interstitial nephritis

45

1.3

1.4

62

1.5

1.6

107

1.4

1.5

Acute tubular necrosis

9

0.3

0.3

10

0.2

0.2

19

0.3

0.2

Dense deposit disease

3

0.1

0.1

5

0.1

0.1

8

0.1

0.1

Others

366

11.0

11.3

487

11.9

12.5

853

11.5

12.0

Total

3,336

100.0

100.0

4,106

100.0

100.0

7,442

100.0

100.0

aPatients classified as either “Renal graft” or “Renal transplantation” in other categories were also excluded

Primary glomerular disease (except IgAN) and nephrotic syndrome in the J-RBR

In the cohort of primary glomerular diseases (except IgA nephropathy) as classified based on the pathogenesis, membranous nephropathy (MN) was predominant in 2009, followed by minor glomerular abnormalities, while minor glomerular abnormalities were the most common diagnosis in 2010, followed by MN (Table 8).
Table 8

The frequency of pathological diagnoses as classified by histopathology in primary glomerular disease except IgA nephropathy in native kidneys in J-RBR 2009 and 2010

Classification

2009

2010

Total

n

%

n

%

n

%

Membranous nephropathy

259

30.1

330

30.3

589

30.2

Minor glomerular abnormalities

216

25.1

408

37.5

624

32.0

Mesangial proliferative glomerulonephritis

167

19.4

86

7.9

253

13.0

Focal segmental glomerulosclerosis

113

13.1

149

13.7

262

13.4

Membranoproliferative glomerulonephritis (types I and III)

48

5.6

51

4.7

99

5.1

Crescentic and necrotizing glomerulonephritis

19

2.2

18

1.7

37

1.9

Endocapillary proliferative

glomerulonephritis

8

0.9

24

2.2

32

1.6

Chronic interstitial nephritis

7

0.8

3

0.3

10

0.5

Sclerosing glomerulonephritis

7

0.8

3

0.3

10

0.5

Nephrosclerosis

5

0.6

7

0.6

12

0.6

Acute interstitial nephritis

1

0.1

0

1

0.1

Acute tubular necrosis

0

1

0.1

1

0.1

Others

11

1.3

9

0.8

20

1.0

Total

861

100.0

1,089

100.0

1,950

100.0

In the patients with nephrotic syndrome as classified by the clinical diagnosis, primary glomerular disease other than IgAN was the predominant diagnosis in both 2009 and 2010, followed by diabetic nephropathy, which was the same order as in 2007 and 2008 (Table 9). Among the patients with primary glomerular diseases (except IgA nephropathy) who had nephrotic syndrome, MN was dominant, followed by minor glomerular abnormalities, viz., minimal change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS), and membranoproliferative glomerulonephritis (MPGN) (types I and III) in 2009. In 2010, minor glomerular abnormalities were the leading diagnosis, followed by MN, FSGS, and MPGN (types I and III) (Table 10).
Table 9

The frequency of pathological diagnoses as classified by pathogenesis in nephrotic syndrome in native kidneys in J-RBR 2009 and 2010

Classification

2009

2010

Total

n

%

n

%

n

%

Primary glomerular disease (except IgA nephropathy)

442

62.3

696

66.7

1,138

64.9

Diabetic nephropathy

85

12.0

78

7.5

163

9.3

IgA nephropathy

30

4.2

36

3.5

66

3.8

Lupus nephritis

30

4.2

58

5.6

88

5.0

Amyloid nephropathy

27

3.8

41

3.9

68

3.9

Infection-related nephropathy

6

0.8

7

0.7

13

0.7

Hypertensive nephrosclerosis

6

0.8

10

0.9

16

0.9

Purpura nephritis

4

0.6

8

0.8

12

0.7

Alport syndrome

3

0.4

0

3

0.2

Thrombotic microangiopathy

1

0.1

1

0.1

2

0.1

PR3-ANCA positive nephritis

1

0.1

0

1

0.1

MPO-ANCA positive nephritis

1

0.1

2

0.2

3

0.2

Others

74

10.4

106

10.2

180

10.3

Total

710

100.0

1,043

100.0

1,753

100.0

MPO myeloperoxidase, ANCA anti-neutrophil cytoplasmic antibody, PR3 proteinase 3

Table 10

The frequency of pathological diagnoses as classified by histopathology in primary glomerular disease except IgA nephropathy in nephrotic syndrome in native kidneys in J-RBR 2009 and 2010

Classification

2009

2010

Total

n

%

n

%

n

%

Membranous nephropathy

178

40.3

227

32.6

405

35.6

Minor glomerular abnormalities

172

38.9

348

50.0

520

45.7

Focal segmental glomerulosclerosis

47

10.6

82

11.8

129

11.3

Membranoproliferative glomerulonephritis (types I and III)

25

5.7

18

2.6

43

3.8

Mesangial proliferative glomerulonephritis

11

2.5

13

1.9

24

2.1

Crescentic and necrotizing glomerulonephritis

2

0.5

2

0.3

4

0.4

Sclerosing glomerulonephritis

2

0.5

0

2

0.2

Endocapillary proliferative glomerulonephritis

1

0.2

5

0.7

6

0.5

Others

4

0.9

1

0.1

5

0.4

Total

442

100.0

696

100.0

1,138

100.0

Clinical diagnosis of membranous nephropathy, minor glomerular abnormalities, and focal segmental glomerulosclerosis in patients with primary glomerular diseases (except IgA nephropathy) in the J-RBR

A subanalysis of the subjects with a clinical diagnosis of MN, minor glomerular abnormalities, and FSGS who had primary glomerular diseases (except IgA nephropathy) was also performed, since these were the most common forms of such diseases. Nephrotic syndrome was the most common clinical diagnosis in cases with primary MN and primary minor glomerular abnormalities (MCNS) (Tables 11, 12), whereas chronic nephritic syndrome and nephrotic syndrome were the most common in cases with primary FSGS in 2009 and 2010, respectively (Table 13).
Table 11

The frequency of clinical diagnoses in membranous nephropathy in primary glomerular disease except IgA nephropathy in native kidneys in J-RBR 2009 and 2010

Classification

2009

2010

Total

n

%

n

%

n

%

Nephrotic syndrome

178

68.7

227

68.8

405

68.8

Chronic nephritic syndrome

74

28.6

93

28.2

167

28.4

Recurrent or persistent hematuria

3

1.2

3

0.9

6

1.0

Renal disorder with collagen disease or vasculitis

1

0.4

1

0.3

2

0.3

Hypertensive nephropathy

1

0.4

0

1

0.2

Rapidly progressive nephritic syndrome

0

1

0.3

1

0.2

Renal disorder with metabolic disease

0

1

0.3

1

0.2

Acute nephritic syndrome

0

1

0.3

1

0.2

Acute renal failure

0

1

0.3

1

0.2

Others

2

0.8

2

0.6

4

0.7

Total

259

100.0

330

100.0

589

100.0

Table 12

The frequency of clinical diagnoses in minor glomerular abnormalities in primary glomerular disease except IgA nephropathy in native kidneys in J-RBR 2009 and 2010

Classification

2009

2010

Total

n

%

n

%

n

%

Nephrotic syndrome

172

79.6

348

85.3

520

83.3

Chronic nephritic syndrome

35

16.2

50

12.3

85

13.6

Recurrent or persistent hematuria

5

2.3

5

1.2

10

1.6

Acute renal failure

1

0.5

0

1

0.2

Rapidly progressive nephritic syndrome

1

0.5

1

0.2

2

0.3

Acute nephritic syndrome

1

0.5

1

0.2

2

0.3

Hypertensive nephropathy

0

1

0.2

1

0.2

Others

1

0.5

2

0.5

3

0.5

Total

216

100.0

408

100.0

624

100.0

Table 13

The frequency of clinical diagnoses in focal segmental glomerulosclerosis in primary glomerular disease except IgA nephropathy in native kidneys in J-RBR 2009 and 2010

Classification

2009

2010

Total

n

%

n

%

n

%

Chronic nephritic syndrome

62

54.9

55

36.9

117

44.7

Nephrotic syndrome

47

41.6

82

55.0

129

49.2

Rapidly progressive nephritic syndrome

1

0.9

1

0.7

2

0.8

Renal disorder with metabolic disease

1

0.9

3

2.0

4

1.5

Recurrent or persistent hematuria

1

0.9

1

0.7

2

0.8

Hypertensive nephropathy

0

2

1.3

2

0.8

Acute nephritic syndrome

0

1

0.7

1

0.4

Inherited renal disease

0

1

0.7

1

0.4

Others

1

0.9

3

2.0

4

1.5

Total

113

100.0

149

100.0

262

100.0

Subanalysis of cases of IgA nephropathy in the J-RBR

The profile, distribution of age ranges, classification of the clinical diagnosis, and the pathological diagnosis of IgAN, the most common glomerulonephritis reported in the J-RBR, were further analyzed (Tables 14, 15, 16, 17, 18, S2, S3). The average age of the overall subjects was in the fourth decade. There were no differences in the proportion of patients based on gender, but the age was significantly higher in males than in females in 2009 (Table 14). In terms of the distribution of age ranges, the peak distribution was in the twenties individually in both genders and in the overall cases in 2009, while it was in the thirties in both genders and overall in 2010, as well as in the combined data from 2009 and 2010 (Table 15). Patients younger than 20 years of age comprised 14.4 % of the cases and those 65 years and over comprised 7.9 % of the cases in the combined data from 2009 and 2010 (Table 15). The majority of the clinical and pathological diagnoses were chronic nephritic syndrome (Table 16) and mesangial proliferative glomerulonephritis (Table 17), respectively, in 2009 and 2010. The distribution of chronic kidney disease (CKD) stages, degree of proteinuria and clinical parameters in IgAN were analyzed in the combined data from 2009 and 2010 (Tables 18, S2, S3).
Table 14

The profile of IgA nephropathy in native kidneys in J-RBR 2009 and 2010

IgA nephropathy

2009

2010

Total

Total native kidney biopsies (n)

1,001

1,176

2,177

 Average age (years)

38.1 ± 17.2

39.3 ± 17.0

38.7 ± 17.1

 Median age (years)

35 (24–52)

38 (26–53)

37 (25–52)

 Male, n (%)

498 (49.8 %)a

585 (49.7 %)

1,083 (49.7 %)

  Average age (years)

39.5 ± 18.2b

40.5 ± 18.4b

40.0 ± 18.3b

  Median age (years)

38 (24–55)b

39 (25–56)

38 (24–56)b

 Female, n (%)

503 (50.2 %)a

591 (50.3 %)

1,094 (50.3 %)

  Average age

36.6 ± 15.9b

38.1 ± 15.4b

37.5 ± 15.7b

  Median age

34 (24–49)b

37 (26–49)

36 (25–49)b

aRatio indicates percentage of each gender in each biopsy category

b P < 0.05 compared to other gender

Table 15

Distribution of age ranges and gender in IgA nephropathy in J-RBR in 2009 and 2010

Age (years)

2009

2010

Total

Male

Female

Total

Male

Female

Total

Male

Female

Total

0–9

11

5

16

12

9

21

23

14

37

10–19

73

68

141

80

55

135

153

123

276

20–29

91

116

207

91

127

218

182

243

425

30–39

87

115

202

113

153

266

200

268

468

40–49

65

81

146

94

106

200

159

187

346

50–59

87

62

149

84

75

159

171

137

308

60–69

62

45

107

82

48

130

144

93

237

70–79

19

9

28

20

18

38

39

27

66

80+

3

2

5

9

0

9

12

2

14

Total

498

503

1,001

585

591

1,176

1,083

1,094

2,177

Under 20 (%)

16.9

14.5

15.7

15.7

10.8

13.3

16.3

12.5

14.4

65 and over (%)

9.4

5.2

7.3

11.5

5.4

8.4

10.5

5.3

7.9

Table 16

The frequency of classification of clinical diagnoses in IgA nephropathy in native kidneys in J-RBR 2009 and 2010

Clinical diagnosis

2009

2010

Total

n

%

n

%

n

%

Chronic nephritic syndrome

886

88.5

1,064

90.5

1,950

89.6

Recurrent or persistent hematuria

49

4.9

40

3.4

89

4.1

Nephrotic syndrome

30

3.0

36

3.1

66

3.0

Rapidly progressive nephritic syndrome

14

1.4

20

1.7

34

1.6

Acute nephritic syndrome

8

0.8

9

0.8

17

0.8

Renal disorder with collagen disease or vasculitis

4

0.4

1

0.1

5

0.2

Acute renal failure

2

0.2

2

0.2

4

0.2

Drug-induced nephropathy

2

0.2

1

0.1

3

0.1

Renal disorder with metabolic disease

1

0.1

0

1

0.0

Hypertensive nephropathy

0

1

0.1

1

0.0

Others

5

0.5

2

0.2

7

0.3

Total

1,001

100.0

1,176

100.0

2,177

100.0

Table 17

The frequency of pathological diagnoses as classified by histopathology in IgAN in native kidneys in J-RBR 2009 and 2010

Pathological diagnosis by histopathology

2009

2010

Total

n

%

n

%

n

%

Mesangial proliferative glomerulonephritis

937

93.6

1,111

94.5

2,048

94.1

Endocapillary proliferative glomerulonephritis

12

1.2

2

0.2

14

0.6

Minor glomerular abnormalities

12

1.2

15

1.3

27

1.2

Focal segmental glomerulosclerosis

9

0.9

6

0.5

15

0.7

Crescentic and necrotizing glomerulonephritis

8

0.8

10

0.9

18

0.8

Nephrosclerosis

6

0.6

4

0.3

10

0.5

Membranous nephropathy

4

0.4

2

0.2

6

0.3

Membranoproliferative glomerulonephritis (types I and III)

4

0.4

5

0.4

9

0.4

Sclerosing glomerulonephritis

3

0.3

2

0.2

5

0.2

Chronic interstitial nephritis

1

0.1

2

0.2

3

0.1

Acute interstitial nephritis

0

1

0.1

1

0.0

Others

5

0.5

16

1.4

21

1.0

Total

1,001

100.0

1,176

100.0

2,177

100.0

Table 18

Distribution of CKD stages and clinical parameters in total in IgA nephropathy in J-RBR: Combined data of 2009 and 2010

 

CKD stage

Total

P value*

 

G1

G2

G3a/b

G4

G5

Total

663

814

551

111

30

2,169

n (%)

30.6

37.5

25.4

5.1

1.4

100.0

Age (years), average

23.5 ± 10.9

40.3 ± 13.5

50.9 ± 13.0

55.7 ± 16.2

46.3 ± 20.4

38.7 ± 17.1

<0.0001

 Median

22 (17–29)

38 (30–50)

52 (42–61)

59 (44–68)

46 (29–62)

37 (25–52)

<0.0001

Body mass index

21.0 ± 4.0

22.9 ± 3.8

23.6 ± 3.7

23.0 ± 4.5

23.4 ± 5.9

22.5 ± 4.0

<0.0001

Estimated GFR (mL/min/1.73 m2)

108.2 (96.9–128.0)

75.2 (67.8–82.7)

49.1 (42.0–54.6)

23.6 (20.9–27.6)

8.5 (6.1–12.0)

74.6 (53.8–95.0)

<0.0001

Proteinuria (g/day)

0.30 (0.10–0.81)

0.50 (0.21–1.00)

0.92 (0.40–2.00)

1.60 (0.71–2.84)

2.81 (1.17–4.58)

0.59 (0.22–1.29)

<0.0001

Proteinuria (g/gCr)

0.39 (0.14–0.91)

0.63 (0.28–1.23)

1.03 (0.51–2.01)

1.69 (0.77–4.21)

2.91 (1.30–4.58)

0.70 (0.27–1.47)

<0.0001

Sediment RBC ≥5/hpf (%)

82.4

81.3

74.6

82.0

86.7

80.0

0.0075

Serum creatinine (mg/dL)

0.60 (0.53–0.70)

0.79 (0.70–0.91)

1.16 (1.00–1.36)

2.10 (1.86–2.47)

5.34 (4.06–7.66)

0.81 (0.65–1.07)

<0.0001

Serum albumin (g/dL)

4.15 ± 0.46

4.02 ± 0.49

3.79 ± 0.59

3.45 ± 0.63

3.22 ± 0.59

3.96 ± 0.56

<0.0001

Serum total cholesterol (mg/dL)

184.6 ± 37.4

204.3 ± 46.2

209.9 ± 51.1

211.6 ± 52.3

221.0 ± 58.6

200.2 ± 46.8

<0.0001

Systolic BP (mmHg)

113.9 ± 14.0

123.3 ± 16.2

130.3 ± 17.5

137.6 ± 22.5

147.5 ± 27.9

123.2 ± 18.1

<0.0001

Diastolic BP (mmHg)

67.6 ± 11.4

75.1 ± 12.3

78.9 ± 12.5

81.0 ± 15.6

87.8 ± 18.0

74.2 ± 13.3

<0.0001

Anti-hypertensive agents (%)

13.8

33.3

59.6

75.8

71.4

37.0

<0.0001

Diabetes mellitus (%)

1.5

3.1

7.7

21.1

0.0

4.6

<0.0001

Data are presented as the mean ± SD or the medians (interquartile ranges)

CKD chronic kidney disease, GFR glomerular filtration rate, RBC red blood cell count, BP blood pressure

* ANOVA, Kruskal–Wallis or χ2-test as appropriate. There are eight (0.4 %) missing values of CKD stage because of inappropriate data for serum creatinine

With regard to the stages of CKD in patients with IgAN, stage 2 was predominant in the combined data from 2009 and 2010 (Table 18) and in both genders (Tables S2 and S3). The degree of proteinuria in the 24-h urine or spot urine samples increased with the progression of CKD stages in the combined data from 2009 and 2010 (Table 18) and in both genders (Tables S2 and S3). The systolic and diastolic blood pressure also increased with the progression of the CKD stage (Tables 18, S2, S3). Overall, 37.0 % of IgAN cases were being treated with anti-hypertensive agents and 4.6 % had diabetes mellitus (Table 18).

Cases in the J-KDR not reported in the J-RBR

In cases in the J-KDR not reported in the J-RBR, a clinical diagnosis of chronic nephritic syndrome was predominant in 2009, followed by hypertensive nephropathy, and a clinical diagnosis of renal disorder with metabolic disease (diabetic nephropathy) was predominant in 2010, followed by nephrotic syndrome (Table 19). Polycystic kidney disease was detected in 2010 as a result of the secondary research studies performed on the basis of the J-KDR as described in the “Subjects and methods” section.
Table 19

The frequency of classification of clinical diagnoses in other 680 cases than J-RBR in J-KDR 2009 and 2010

Classification

Other cases 2009 (n = 680)

Other cases 2010 (n = 575)

Total (n = 1,255)

n

%

n

%

n

%

Chronic nephritic syndrome

165

24.3

72

12.5

237

18.9

Hypertensive nephropathy

142

20.9

43

7.5

185

14.7

Renal disorder with metabolic disease

106

15.6

177

30.8

283

22.5

Nephrotic syndrome

86

12.6

118

20.5

204

16.3

Renal disorder with collagen disease or vasculitis

24

3.5

7

1.2

31

2.5

Rapidly progressive nephritic syndrome

21

3.1

18

3.1

39

3.1

Inherited renal disease

18

2.6

3

0.5

21

1.7

Acute renal failure

9

1.3

10

1.7

19

1.5

Recurrent or persistent hematuria

8

1.2

0

8

0.6

Acute nephritic syndrome

5

0.7

4

0.7

9

0.7

Drug-induced nephropathy

5

0.7

0

5

0.4

Renal transplantation

2

0.3

9

1.6

11

0.9

Polycystic kidney disease

82

14.3

82

6.5

Others

89

13.1

32

5.6

121

9.6

Total

680

100.0

575

100.0

1,255

100.0

Secondary and longitudinal research by the J-RBR/J-KDR

Five of the secondary and longitudinal research studies, viz., the JNSCS, J-IDCS, J-IGACS, JRPGN-CS, and JDNCS, were started in 2009, and the J-PKD was started in 2010 in association with the J-RBR/J-KDR.

Discussion and comments

In 2009, the J-KDR started to register clinically-diagnosed cases without renal biopsies, in addition to cases with renal biopsies included in the J-RBR, which had been started in 2007. More than 80 % of the registered cases were in the J-RBR in 2009 and 2010, and thus the detailed data from the J-RBR and the clinical diagnosis alone for the J-KDR are described in this report.

The rates of primary glomerular disease (except IgAN) combined with that of IgAN in native renal biopsies were 59.3 %, 56.5 %, 58.8 %, and 58.5 % in 2007, 2008, 2009, and 2010 in the J-RBR. A recent report from a single center in Japan gave the rates as 77.8 % and 75.9 % between 1979 and 2008 and between 2004 and 2008, respectively [5]. In the present report for the J-RBR, the peak distribution of age was in the sixties in the combined data for 2009 and 2010. The difference in the rates of primary glomerular disease including IgAN may have been due to the higher mean ages of native biopsy cases in the J-RBR compared to the single center in this period (mean age, 46.7 vs. 40.8 years; age of the peak number, sixties vs. twenties), because the incidence of secondary glomerular disease increases in elderly patients, as reported previously [5].

IgAN is still the most frequently diagnosed disease in native kidney biopsies in Japan (33.0 %, 30.2 %, 31.6 %, and 30.4 % of cases in 2007, 2008, 2009, and 2010 in the J-RBR) [1, 4, 5, 6] similar to other Asian countries [7, 8] and some European countries [9, 10]. The peak distribution of age ranges was the twenties in 2009 and thirties in 2010. In patients with IgAN, the majority (68.1 %) of renal biopsies were performed in CKD stages G1 and G2, with median proteinuria less than 1 g per day (Table 18), suggesting that there was a relatively early diagnosis of this biopsy-proven disease.

In the present clinical data, the degree of proteinuria increased with the progression of the CKD stage, and was more than 1 g per day for the median value in patients with CKD stages G4 and G5 (Tables 18, S1, S2). Previously, the best single predictor for renal deterioration was severe proteinuria on urine dipstick testing (≥100 mg/dL), followed by hypoalbuminemia, mild hematuria, serum total protein levels, diastolic blood pressure, and histological grade, in a cohort study with 10 years follow-up from 1995 in Japan, the cohort of which exhibited a younger median age (27.7 years) and a peak distribution of age ranges in the teens [11, 12]. A recent report suggested that IgAN with nephrotic syndrome had a worse renal outcome compared to IgAN with non-nephrotic syndrome unless partial or complete remission was achieved [13]. Further studies are necessary to elucidate the risk factors or predictors for renal deterioration in IgAN in the present era utilizing the J-RBR, possibly as part of a new secondary clinical study.

MN was the most common histopathology in terms of primary glomerular disease other than IgAN in 2007 (31.4 %), 2008 (25.7 %), and 2009 (30.1 %) in the J-RBR and was also the most common type in primary nephrotic syndrome in 2007 (44.0 %) and 2009 (40.3 %) in the J-RBR. MN was also the most common primary cause of nephrotic syndrome in a northern European Caucasian population, with a biopsy rate of 4.5 per million population per year [14]. A total of 68.7 % and 68.8 % of primary MN cases exhibited nephrotic syndrome as the clinical diagnosis at the time of renal biopsy in 2009 and 2010 in the J-RBR. Yokoyama et al. recently reported in their clinical data analysis of 501 cases collected from the combined data of the J-RBR from 2007 to 2010 that nearly half of primary MN (49.1 %) cases showed a daily proteinuria of 3.5 g or higher [15]. The renal survival rate was 60 % at 20 years after diagnosis in patients with primary MN, and the renal survival rate in patients on steroid therapy was significantly higher in patients on supportive therapy alone in Japan [16], while spontaneous remission was reported to be common (32 %) in patients with primary MN with nephrotic syndrome in Spain [17], even in patients exhibiting chronic renal impairment [18]. Whether treatment with renin–angiotensin blockers or immunoglobulins other than steroids has a favorable effect on the renal prognosis of primary MN should be elucidated in future clinical studies.

The minor glomerular abnormalities in primary nephrotic syndrome, which correspond to MCNS, was the most common histopathology reported in 2008 (44.1 %) and 2010 (50.0 %) in the J-RBR. Since MCNS develops in patients at younger ages [5, 15] while primary MN develops in a relatively elderly population [15, 16], the frequency of these diseases may depend on the distribution of the age ranges of patients registered in each year. Indeed, the rate of native biopsies of subjects younger than 20 years of age slightly increased from 11.4 % in 2009 to 12.7 % in 2010 (Table 3) and the mean age of patients with nephrotic syndrome slightly decreased from 53.5 years in 2009 to 50.1 years in 2010 (Table 5) in the J-RBR.

The average age of rapidly progressive nephritic syndrome was the highest (64.4 years) in the age distribution in the classification of clinical diagnosis in the J-RBR (Table 5). Elderly subjects (65 years and over) comprised nearly 25 % of cases, and very elderly subjects (80 years and over) comprised 2.5 % of the cases in the combined data for 2009 and 2010 in the J-RBR. It has been reported that there were statistically significant differences in the renal disease spectrum between elderly and younger subjects [19, 20]. The frequency of rapidly progressive nephritic syndrome in the clinical diagnosis dramatically increased from 4.0 % in the younger group (20–64 years) to 19.6 % in the very elderly in the combined data from 2007 to November 2011 in the J-RBR [20]. A nationwide survey of rapidly progressive glomerulonephritis (RPGN) was conducted between 1989 and 2007 in Japan, and showed that 64.0 % of patients had pauci-immune-type RPGN, including 42.0 % renal-limited vasculitis, 19.4 % microscopic polyangiitis, and 2.6 % Wegener’s granulomatosis (currently granulomatosis with polyangiitis) [21]. Since the frequency of myeloperoxidase–anti-neutrophil cytoplasmic antibody (MPO-ANCA)-positive nephritis has increased recently [22], a further subanalysis of rapidly progressive nephritic syndrome in the J-RBR should be performed to validate the recently published Japanese guidelines for RPGN [23].

Five new secondary research studies of the J-KDR were started in 2009, viz., the J-NSCS, J-IDCS, J-IGACS, J-RPGNCS, and J-DNCS, and the J-PKD was started in 2010. The J-RBR and J-KDR initiated two more clinical research studies (J-RBR201001 and J-KDR201001) being performed by members of the JSN who had already participated in the registry and who registered cases under the precise regulations presented on the website of the JSN in 2011.

With regard to estimating the number of yearly native renal biopsies in Japan, the Research Group on Progressive Renal Disease from the Ministry of Health, Labor and Welfare of Japan recently reported by a questionnaire method that it was between 18,000 and 21,000 in 2010. The J-RBR may cover nearly one fourth to one fifth of the number of yearly native renal biopsies in Japan in 2010. Since 128,057,352 people resided in Japan in 2010, the estimated rate of renal biopsy was 140.6 to 164.0 per million population. This rate was higher than that in Romania [24], Spain [25], the Czech Republic [10], Denmark [26], and Scotland [27], was similar to that in France [28], and was lower than that in USA, Finland [29], and Australia [30].

There are some limitations in the J-RBR and J-KDR. The J-RBR records three diagnoses for each case, viz., the clinical diagnosis, diagnosis based on the pathogenesis, and the diagnosis based on a histopathological examination, so there may be still some inconsistency in the case records. The terms hypertensive nephropathy, hypertensive nephrosclerosis, nephrosclerosis, and diabetic nephropathy may need to be defined more precisely to improve the accuracy of the report by the J-RBR. The incidence of renal biopsy and the incidence of biopsy-proven renal diseases such as IgAN and primary glomerular disease (except IgAN) could be surveyed in major renal centers in Japan in terms of the epidemiological aspects to work out appropriate countermeasures. In this aspect, the incidence of pediatric IgAN was reported to be 4.5 cases/year per 100,000 children under 15 years of age from 1983 to 1999 in Yonago City, Japan [31], although center variations in the country in terms of the incidence, indications and diagnosis of adult native renal biopsy have been reported [27].

Finally, a committee report of J-KDR including J-RBR in 2009, 2010 and their total was conducted. The J-RBR exhibited the majority of the registry system to elucidate yearly demographic data of renal biopsies in Japan, and J-KDR was utilized to promote advanced clinical research in the field of nephrology in our country.

Notes

Acknowledgments

The authors greatly acknowledge the help and assistance of many colleagues in centers and affiliate hospitals with collection of data for the J-RBR/J-KDR. We also sincerely thank Ms. M. Irie of the UNIN-INDICE and Ms. Y. Saito of the JSN for supporting the registration system and Ms. K. Fukuda of the JSN for submitting the manuscript. 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.

Conflict of interest

T. Saito was supported by Novartis Pharma Co. and Kyowa-Kirin Co. Other authors declare no competing interests.

Supplementary material

10157_2012_746_MOESM1_ESM.doc (98 kb)
Supplementary material 1 (DOC 98 kb)

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Copyright information

© Japanese Society of Nephrology 2013

Authors and Affiliations

  • Hitoshi Sugiyama
    • 1
  • Hitoshi Yokoyama
    • 2
    Email author
  • Hiroshi Sato
    • 3
  • Takao Saito
    • 4
  • Yukimasa Kohda
    • 5
    • 30
  • Shinichi Nishi
    • 6
  • Kazuhiko Tsuruya
    • 7
  • Hideyasu Kiyomoto
    • 8
  • Hiroyuki Iida
    • 9
  • Tamaki Sasaki
    • 10
  • Makoto Higuchi
    • 11
  • Motoshi Hattori
    • 12
  • Kazumasa Oka
    • 13
  • Shoji Kagami
    • 14
  • Tetsuya Kawamura
    • 15
  • Tetsuro Takeda
    • 16
    • 31
  • Hiroshi Hataya
    • 17
  • Yuichiro Fukasawa
    • 18
  • Atsushi Fukatsu
    • 19
    • 32
  • Kunio Morozumi
    • 20
  • Norishige Yoshikawa
    • 21
  • Akira Shimizu
    • 22
  • Hiroshi Kitamura
    • 23
  • Yukio Yuzawa
    • 24
  • Seiichi Matsuo
    • 25
  • Yutaka Kiyohara
    • 26
  • Kensuke Joh
    • 27
  • Michio Nagata
    • 28
  • Takashi Taguchi
    • 29
    • 33
    Email author
  • Hirofumi Makino
    • 1
  • Committee for Standardization of Renal Pathological Diagnosis and Committee for Kidney Disease Registry, Japanese Society of Nephrology, 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.Division of Nephrology and Kidney CenterKobe University School of MedicineKobeJapan
  7. 7.Department of Integrated Therapy for Chronic Kidney Disease, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  8. 8.Division of Integrated Nephrology and Telemedicine, Department of Community Medical SupportsTohoku Medical Megabank Organization, Tohoku UniversitySendaiJapan
  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 PathologyHyogo Prefectural Nishinomiya HospitalHyogoJapan
  14. 14.Department of PediatricsThe Institute of Health Bioscience, The University of Tokushima Graduate SchoolTokushimaJapan
  15. 15.Department of Medicine, Division of Kidney and HypertensionJikei University School of MedicineTokyoJapan
  16. 16.Division of Clinical Nephrology and RheumatologyNiigata University Graduate School of Medical and Dental SciencesNiigataJapan
  17. 17.Department of NephrologyTokyo Metropolitan Children’s Medical CenterTokyoJapan
  18. 18.Department of PathologyKKR Sapporo Medical CenterSapporoJapan
  19. 19.Department of NephrologyKyoto University Graduate School of MedicineKyotoJapan
  20. 20.Kidney Center, Japanese Red Cross Nagoya Daini HospitalNagoyaJapan
  21. 21.Department of PediatricsWakayama Medical University, School of MedicineWakayamaJapan
  22. 22.Department of PathologyNippon Medical SchoolTokyoJapan
  23. 23.Department of Pathology, Clinical Research CenterNational Hospital Organization Chiba East National HospitalChibaJapan
  24. 24.Department of NephrologyFujita Health University School of MedicineToyoakeJapan
  25. 25.Department of NephrologyNagoya University Graduate School of MedicineNagoyaJapan
  26. 26.Department of Environmental Medicine, Graduate School of Medical SciencesKyushu UniversityFukuokaJapan
  27. 27.Division of PathologySendai Shakai Hoken HospitalSendaiJapan
  28. 28.Molecular Pathology, Biomolecular and Integrated Medical SciencesGraduate School of Comprehensive Human Sciences, University of TsukubaTsukubaJapan
  29. 29.Department of PathologyNagasaki University Graduate School of Biomedical SciencesNagasakiJapan
  30. 30.Hikarinomori ClinicKumamotoJapan
  31. 31.Department of NephrologyDokkyo Medical University Koshigaya HospitalKoshigayaJapan
  32. 32.Department of NephrologyYachiyo HospitalAnjoJapan
  33. 33.Department of PathologyNagasaki Municipal HospitalNagasakiJapan

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