Dataset Information

The revised version 2018 of the nationwide web-based registry system for kidney diseases in Japan: Japan Renal Biopsy Registry and Japan Kidney Disease Registry.

ABSTRACT:

Background

The Japan Renal Biopsy Registry (J-RBR), the first nation-wide registry of renal biopsies in Japan, was established in 2007, and expanded to include non-biopsy cases as the Japan Kidney Disease Registry (J-KDR) in 2009. The J-RBR/J-KDR is one of the biggest registries for kidney diseases. It has revealed the prevalence and distribution of kidney diseases in Japan. This registry system was meant to be revised after 10 years.

Methods

In 2017, the Committees of the Japanese Society of Nephrology started a project for the revision of the J-RBR/J-KDR. The revised system was designed in such a way that the diagnoses of the patients could be selected from the Diagnosis Panel, a list covering almost all known kidney diseases, and focusing on their pathogenesis rather than morphological classification. The Diagnosis Panel consists of 22 categories (18 glomerular, 1 tubulointerstitial, 1 congenital/genetical, 1 transplant related, and 1 other) and includes 123 diagnostic names. The items for clinical diagnosis and laboratory data were also renewed, with the addition of the information on immunosuppressive treatment.

Results

The revised version of J-RBR/J-KDR came into use in January 2018. The number of cases registered under the revised system was 2748 in the first year. The total number of cases has reached to 43,813 since 2007.

Conclusion

The revised version 2018 J-RBR/J-KDR system attempts to cover all kidney diseases by focusing on their pathogenesis. It will be a new platform for the standardized registration of kidney biopsy cases that provides more systemized data of higher quality.

SUBMITTER: Ozeki T

PROVIDER: S-EPMC7524691 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

The revised version 2018 of the nationwide web-based registry system for kidney diseases in Japan: Japan Renal Biopsy Registry and Japan Kidney Disease Registry.

Ozeki Takaya T Maruyama Shoichi S Nagata Michio M Shimizu Akira A Sugiyama Hitoshi H Sato Hiroshi H Yokoyama Hitoshi H

Clinical and experimental nephrology 20200806 11

<h4>Background</h4>The Japan Renal Biopsy Registry (J-RBR), the first nation-wide registry of renal biopsies in Japan, was established in 2007, and expanded to include non-biopsy cases as the Japan Kidney Disease Registry (J-KDR) in 2009. The J-RBR/J-KDR is one of the biggest registries for kidney diseases. It has revealed the prevalence and distribution of kidney diseases in Japan. This registry system was meant to be revised after 10 years.<h4>Methods</h4>In 2017, the Committees of the Japanes ...[more]

PMID: 32761468

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Project description:IntroductionThe Japan Renal Biopsy Registry (J-RBR) was started in 2007 by the Committee for the 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 clarify drug-induced kidney disease (DIKD) of renal biopsied cases in Japan.Subjects and methodsWe analyzed the data of 26,535 cases that were registered in the J-RBR from 2007 to 2015.ResultsBased on clinical and pathological diagnoses, 328 cases (176 males and 152 females) of renal biopsy-proven DIKD were registered in the J-RBR from 2007 to 2015 (1.24 % of all cases). The frequency of DIKD increased with age. The number of cases peaked in the 6th-8th decade in all pathological categories, except for the number of chronic tubulointerstitial lesions (CTIL), which peaked in the 4th-5th decade. Overall, the frequency of DIKD was 3 times higher in the 7th decade than in the 2nd decade (1.86 vs. 0.62 %). The main clinical diagnoses were DIKD in 150 cases (45.7 %), nephrotic syndrome in 66 cases (20.1 %), chronic nephritic syndrome in 55 cases (16.8 %), and rapidly progressive glomerulonephritis in 30 cases (9.1 %). DIKD was registered as a secondary diagnosis in 136 cases (41.5 %). The pathological findings of these cases were glomerular lesions in 105 cases (32.0 %), acute tubulointerstitial lesions (ATIL) in 87 cases (26.5 %), CTIL in 72 cases (22.0 %), and sclerotic glomerular lesions and/or nephrosclerosis in 18 cases (5.5 %). ATIL and CTIL were mainly found in cases in which DIKD was diagnosed on the basis of the patient's clinical findings. In addition, nephrotic syndrome-related membranous nephropathy (MN) was the major cause of renal damage in 59.4 % of the cases involving glomerular injuries. According to the CGA risk classification, high-risk (red zone) cases accounted for 56.1 % of all cases of DIKD and 75.9, 64.9, and 33.3 % of the cases involving ATIL, CTIL, and glomerular injuries, respectively. The causative drugs were identified in 102 cases, including bucillamine in 38 cases of MN, gemcitabine in 3 cases of thrombotic microangiopathy, and other anticancer drugs in 14 cases (anti-vascular endothelial growth factor drugs in 3 cases and propyl thiouracil in 3 cases of anti-neutrophil cytoplasmic antibody-related nephritis).ConclusionOur analysis of the J-RBR revealed that DIKD mainly affects elderly people in Japan. ATIL or CTIL were found in approximately half of the biopsied cases of DIKD, and one-third involved glomerular lesions, mainly MN or clinical nephrotic syndrome.

Project description:BackgroundKidney complications associated with anticancer drug therapy have greatly increased recently. We aimed to investigate the real-world clinical outcomes of anticancer drug therapy-associated renal complications in Japan using the national kidney biopsy database, Japan Renal Biopsy Registry (J-RBR).MethodsFrom 2018 to 2021, 449 cases from 49 facilities identified as 'drug-induced' histopathology in the J-RBR were screened, of which a total of 135 were confirmed as anticancer drug-related cases and included in the analysis. Overall survival rates were estimated using the Kaplan-Meier method and compared by logrank test. The Cox regression model was used to evaluate the association between variables and deaths.ResultsThe most common primary sites of malignancies were the lung (33.3%), followed by gastrointestinal (16.3%) and gynaecological (11.1%) cancers. Tubulointerstitial nephritis (TIN; 47.4%) and thrombotic microangiopathy (TMA; 35.6%) were the most frequent diagnoses. All immunoglobulin A nephropathy, minimal change disease and crescentic glomerulonephritis (CrGN) cases were immune checkpoint inhibitor related. All CrGN cases were anti-neutrophil cytoplasmic antibody negative. Antibiotics were most frequently used concomitantly with anticancer drugs in TMA cases among subgroups (TMA versus others: 62.5 versus 27.5%; P < .001). Among TMA cases, the serum lactate dehydrogenase level tended to be higher in cytotoxic agent-associated TMA (CTx-TMA) than in other TMAs, but was not significant between groups (415.5 versus 219.0 U/l; P = .06). Overall survival was worse in CTx-TMA than in other TMAs (P = .007). The Cox model demonstrated proton pump inhibitor (PPI) use (hazard ratio 2.49, P = .001) as a significant prognostic factor, as well as the presence of metastasis and serum albumin level.ConclusionsOur registry analysis highlighted various presentations of biopsy-proven kidney complications associated with anticancer drug therapy. Clinicians should be aware of worse outcomes associated with CTx-TMA and the prognostic role of PPI use.

Project description:BackgroundThe clinical presentation and prognosis of adult and elderly patients with IgA vasculitis (Henoch-Schönlein purpura) accompanied by nephritis (IgAV-N) have not been investigated in detail. We therefore surveyed the features and outcomes of IgAV-N based on nationwide data derived from the Japan Renal Biopsy Registry (J-RBR).MethodsThis multi-center cohort study compared the clinicopathological parameters at diagnosis, initial therapies and outcomes between 106 adult (age 19-64 years) and 46 elderly (≥65 years) patients with IgAV-N who were registered in the J-RBR between 2007 and 2012. The primary end-points comprised a 50% increase in serum creatinine (sCr) values or end-stage kidney disease. Factors affecting a decrease in renal function were assessed using Cox proportional hazards models.ResultsRates of hypertension, impaired renal function, hypoalbuminemia and crescentic glomerulonephritis were significantly higher among the elderly, than the adult patients. About 80% and 60% of the patients in both groups were respectively treated with corticosteroid and a renin-angiotensin system (RAS) blockade. Both groups had favorable renal survival rates for nine years (93.6% and 91.4% of the adult and elderly patients, respectively). Significantly more elderly than adult patients developed a 50% increase in sCr during a mean observation period of 3.9 years (21.7% vs. 4.7%, p = 0.012), and significantly fewer elderly, than adult patients achieved clinical remission (23.9% vs. 46.2%, p = 0.016). Multivariate analysis selected advanced age (≥65 years) and lower serum albumin values as independent prognostic factors for a decline in renal function, whereas steroid pulse therapy helped to preserve renal function.ConclusionsThe renal prognosis of adult and elderly patients with IgAV-N was favorable when treated aggressively with corticosteroid and RAS blockade. However, the course of renal function should be carefully monitored in patients aged over 65 years and those with hypoalbuminemia.

Dataset Information

The revised version 2018 of the nationwide web-based registry system for kidney diseases in Japan: Japan Renal Biopsy Registry and Japan Kidney Disease Registry.

Background

Methods

Results

Conclusion

Publications

The revised version 2018 of the nationwide web-based registry system for kidney diseases in Japan: Japan Renal Biopsy Registry and Japan Kidney Disease Registry.

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