Abstract:We have shown that 18.6% of patients with assumed benign IgAN had progressive disease after a median duration of 22 years and that these patients could not be predicted at the time of biopsy. Our study demonstrates that an extended follow-up period is needed when assessing prognosis in this group of patients.
“…Although long-term renal prognoses of kidney diseases are clinically relevant, there is no established histopathological factor to predict the long-term renal prognosis of IgA nephropathy (IgAN). Knoop et al used data from the Norwegian Kidney Biopsy Registry and found that none of the histopathological variables at the time of biopsy could identify patients with long-term progressive disease [1]. Coppo et al conducted long-term follow-up analyses of the original Validation Study of the Oxford Classification for IgA Nephropathy cohort, and showed an independent relationship between kidney biopsy findings and the risk of progression towards kidney failure in patients with IgAN.…”
There is no effectual pathological factor to predict the long-term renal prognosis of IgA nephropathy. Glomerular hypertrophy plays a crucial role in kidney disease outcomes in both experimental models and humans. This study aimed to 1) confirm the long-term prognostic significance of a maximal glomerular diameter (Max GD) � 242.3 μm, 2) test a renal prognosis prediction model adding Max GD � 242.3 μm to the Oxford classification (MEST-C), and 3) examine the time series changes in the long-term renal prognosis of patients with IgA nephropathy. The study included 43 patients diagnosed with IgA nephropathy from 1993 to 1998 at Kameda General Hospital. Renal prognosis with the endpoint of a 50% reduction in estimated glomerular filtration rate (eGFR) or the development of end-stage renal disease requiring dialysis was examined using logistic regression analysis, Cox regression analysis, and the Kaplan-Meier method. Pathological evaluation was performed using MEST-C and Max GD, and the validity of the prediction model was evaluated. Patients with Max GD � 242.3 μm had significantly poor renal prognosis with multivariate Cox analysis (P = 0.0293). The results of the Kaplan-Meier analysis showed that kidney survival rates in the high-Max GD group were significantly lower than those in the low-Max GD group (log rank, P = 0.0043), which was confirmed in propensity score-matched models (log rank, P = 0.0426). Adding Max GD � 242.3 μm to MEST-C improved diagnostic power of the renal prognosis prediction model by renal pathology tissue examination (R 2 : 3.3 to 14.5%, AICc: 71.8 to 68.0, C statistic: 0.657 to 0.772). We confirm that glomerular hypertrophy is useful as a long-term renal prognostic factor.
“…Although long-term renal prognoses of kidney diseases are clinically relevant, there is no established histopathological factor to predict the long-term renal prognosis of IgA nephropathy (IgAN). Knoop et al used data from the Norwegian Kidney Biopsy Registry and found that none of the histopathological variables at the time of biopsy could identify patients with long-term progressive disease [1]. Coppo et al conducted long-term follow-up analyses of the original Validation Study of the Oxford Classification for IgA Nephropathy cohort, and showed an independent relationship between kidney biopsy findings and the risk of progression towards kidney failure in patients with IgAN.…”
There is no effectual pathological factor to predict the long-term renal prognosis of IgA nephropathy. Glomerular hypertrophy plays a crucial role in kidney disease outcomes in both experimental models and humans. This study aimed to 1) confirm the long-term prognostic significance of a maximal glomerular diameter (Max GD) � 242.3 μm, 2) test a renal prognosis prediction model adding Max GD � 242.3 μm to the Oxford classification (MEST-C), and 3) examine the time series changes in the long-term renal prognosis of patients with IgA nephropathy. The study included 43 patients diagnosed with IgA nephropathy from 1993 to 1998 at Kameda General Hospital. Renal prognosis with the endpoint of a 50% reduction in estimated glomerular filtration rate (eGFR) or the development of end-stage renal disease requiring dialysis was examined using logistic regression analysis, Cox regression analysis, and the Kaplan-Meier method. Pathological evaluation was performed using MEST-C and Max GD, and the validity of the prediction model was evaluated. Patients with Max GD � 242.3 μm had significantly poor renal prognosis with multivariate Cox analysis (P = 0.0293). The results of the Kaplan-Meier analysis showed that kidney survival rates in the high-Max GD group were significantly lower than those in the low-Max GD group (log rank, P = 0.0043), which was confirmed in propensity score-matched models (log rank, P = 0.0426). Adding Max GD � 242.3 μm to MEST-C improved diagnostic power of the renal prognosis prediction model by renal pathology tissue examination (R 2 : 3.3 to 14.5%, AICc: 71.8 to 68.0, C statistic: 0.657 to 0.772). We confirm that glomerular hypertrophy is useful as a long-term renal prognostic factor.
“…It is noteworthy how old many registries are. These old registries allow for studies about the long-term course of kidney disease and underline the usefulness of kidney biopsy registries [ 44 , 45 ]. The age of the registries is also reflected by the coding systems and mappings used.…”
Background
Kidney biopsy registries all over the world benefit research, teaching and health policy. Comparison, aggregation and exchange of data is however greatly dependent on how registration and coding of kidney biopsy diagnoses are performed. This paper gives an overview over kidney biopsy registries, explores how these registries code kidney disease and identifies needs for improvement of coding practice.
Methods
A literature search was undertaken to identify biopsy registries for medical kidney diseases. These data were supplemented with information from personal contacts and from registry websites. A questionnaire was sent to all identified registries, investigating age of registries, scope, method of coding, possible mapping to international terminologies as well as self-reported problems and suggestions for improvement.
Results
Sixteen regional or national kidney biopsy registries were identified, of which 11 were older than 10 years. Most registries were located either in Europe (10/16) or in Asia (4/16). Registries most often use a proprietary coding system (12/16). Only a few of these coding systems were mapped to SNOMED CT (1), older SNOMED versions (2) or ERA-EDTA PRD (3). Lack of maintenance and updates of the coding system was the most commonly reported problem.
Conclusions
There were large gaps in the global coverage of kidney biopsy registries. Limited use of international coding systems among existing registries hampers interoperability and exchange of data. The study underlines that the use of a common and uniform coding system is necessary to fully realize the potential of kidney biopsy registries.
“…The 10-year renal survival rate reported by different registries varies between 60% and 95%, while up to 50% may reach end-stage renal disease (ESRD) within 20 years of diagnosis [4,5,6]. Moreover, even those patients initially considered to have a “benign” disease show a progressive decline of renal function if followed for more than 20 years [7]. Regional environmental factors, differences in racial composition, and genetic susceptibility are important contributors to IgAN epidemiology and might explain disease susceptibility, heterogeneous presentation, and the risk of progression [8].…”
Immunoglobulin A nephropathy (IgAN) is the most frequent glomerular disease worldwide and a leading cause of end-stage renal disease. Particularly challenging to the clinician is the early identification of patients at high risk of progression, an estimation of the decline in renal function, and the selection of only those that would benefit from additional immunosuppressive therapies. Nevertheless, the pathway to a better prognostication and to the development of targeted therapies in IgAN has been paved by recent understanding of the genetic and molecular basis of this disease. Merging the data from the Oxford Classification validation studies and prospective treatment studies has suggested that a disease-stratifying algorithm would be appropriate for disease management, although it awaits validation in a prospective setting. The emergence of potential noninvasive biomarkers may assist traditional markers (proteinuria, hematuria) in monitoring disease activity and treatment response. The recent landmark trials of IgAN treatment (STOP-IgAN and TESTING trials) have suggested that the risks associated with immunosuppressive therapy outweigh the benefits, which may shift the treatment paradigm of this disease. While awaiting the approval of the first therapies for IgAN, more targeted and less toxic immunotherapies are warranted. Accordingly, the targeting of complement activation, the modulation of mucosal immunity, the antagonism of B-cell activating factors, and proteasomal inhibition are currently being evaluated in pilot studies for IgAN treatment.
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