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      Persistent Disease Activity in Patients With Long-Standing Glomerular Disease

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          Abstract

          Introduction

          Glomerular diseases are characterized by variable disease activity over many years. We aimed to analyze the relationship between clinical disease activity and duration of glomerular disease.

          Methods

          Disease activity in adults with chronic minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, and IgA nephropathy (IgAN; first diagnostic biopsy >5 years before enrollment; Of Longstanding Disease [OLD] cohort, n = 256) followed at Columbia University Medical Center (CUMC), was compared with disease activity of an internal and external cohort of patients with first diagnostic biopsy <5 years before enrollment drawn from the Cure Glomerulonephropathy Network (CureGN cohort, n = 1182; CUMC-CureGN cohort, n = 362). Disease activity was defined by (i) Kidney Disease: Improving Global Outcomes–recommended threshold criteria for initiation of immunosuppression in primary glomerulonephropathy (GN) and (ii) CureGN’s Disease Activity Working Group definitions for activity.

          Results

          No significant differences were detected among the 3 cohorts in terms of age, sex, serum creatinine, and urinary protein-to-creatinine ratio. For each GN subtype, disease activity in the OLD cohort was comparable with disease activity in the entire CureGN and the CUMC-CureGN cohort. When limiting our comparisons to disease activity in incident CUMC-CureGN patients (first diagnostic biopsy within 6 months of enrollment), OLD patients demonstrated similar activity rates as incident patients.

          Conclusion

          Disease activity did not differ among patients with shorter versus longer duration of disease. Such survivor patients, with long-term but persistent disease, are potentially highly informative for understanding the clinical course and pathogenesis of GN and may help identify factors mediating more chronic subtypes of disease.

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          Most cited references23

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          Remission of proteinuria improves prognosis in IgA nephropathy.

          Proteinuria has been shown to be an adverse prognostic factor in IgA nephropathy. The benefit of achieving a partial remission of proteinuria, however, has not been well described. We studied 542 patients with biopsy-proven primary IgA nephropathy in the Toronto Glomerulonephritis Registry and found that glomerular filtration rate (GFR) declined at -0.38 +/- 0.61 ml/min per 1.73 m2/mo overall, with 30% of subjects reaching end-stage renal disease. Multivariate analysis revealed that proteinuria during follow-up was the most important predictor of the rate of GFR decline. Among the 171 patients with 3 g/d (n = 121) lost renal function 25-fold faster than those with or =3 g/d who achieved a partial remission (<1 g/d) had a similar course to patients who had < or =1 g/d throughout, and fared far better than patients who never achieved remission. These results underscore the relationship between proteinuria and prognosis in IgA nephropathy and establish the importance of remission.
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            Prognosis in IgA Nephropathy: 30-Year Analysis of 1,012 Patients at a Single Center in Japan

            Background Little is known about the long-term prognosis of patients with IgA nephropathy (IgAN). Methods This retrospective cohort analysis evaluated clinical and histological findings at the time of renal biopsy, initial treatment, patient outcomes over 30 years, and risk factors associated with progression in 1,012 patients diagnosed with IgAN at our center since 1974. Results Of the 1,012 patients, 40.5% were male. Mean patient age was 33±12 years and mean blood pressure was 122±17/75±13 mmHg. Mean serum creatinine concentration was 0.89±0.42 mg/dL, and mean estimated glomerular filtration rate (eGFR) was 78.5±26.2 ml/min/1.73 m2. Mean proteinuria was 1.19±1.61 g/day, and mean urinary red blood cells were 36.6±35.3/high-powered field. Histologically, mesangial hypercellularity was present in 47.6% of patients, endothelial hypercellularity in 44.3%, segmental sclerosis in 74.6%, and tubular atrophy/interstitial fibrosis in 28.8% by Oxford classification. Initial treatment consisted of corticosteroids in 26.9% of patients, renin-angiotensin-aldosterone system inhibitor in 28.9%, and tonsillectomy plus steroids in 11.7%. The 10-, 20-, and 30-year renal survival rates were 84.3, 66.6, and 50.3%, respectively. Tonsillectomy plus steroids dramatically improved renal outcome. Cox multivariate regression analysis showed that higher proteinuria, lower eGFR, and higher uric acid at the time of renal biopsy were independent risk factors for the development of end stage renal disease (ESRD). Conclusions IgAN is not a benign disease, with about 50% of patients progressing to ESRD within 30 years despite treatment.
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              Focal and segmental glomerulosclerosis: definition and relevance of a partial remission.

              Focal and segmental glomerulosclerosis (FSGS) is one of the most common primary glomerular diseases to terminate in ESRD. A complete remission (CR) confers an excellent long-term prognosis, but the quantitative benefits of partial remissions (PR) have not been defined. This study evaluated the rate of renal function decline (slope of creatinine clearance) and renal survival in nephrotic FSGS patients with CR, PR, or no remission. It also examined relapse rate from remission and its impact on outcome. Multivariate analysis included clinical and laboratory data at presentation and over follow-up, BP control, the agents used, and immunosuppressive therapy. The study cohort was 281 nephrotic FSGS patients who had a minimum of 12 mo of observation and were identified from the Toronto Glomerulonephritis Registry. Over a median follow-up of 65 mo, 55 experienced a CR, 117 had a PR, and 109 had no remission. A PR was independently predictive of slope and survival from renal failure by multivariate analysis (adjusted time-dependent hazard ratio, 0.48; 95% confidence interval, 0.24 to 0.96; P = 0.04). Immunosuppression with high-dose prednisone was associated with a higher rate of PR and CR. Relapse from PR was frequent (56%) and associated with a more rapid rate of renal function decline and worse renal survival compared with relapse-free partial remitters. Only female gender and the nadir of proteinuria during remission were associated with a sustained remission. A PR in proteinuria and its maintenance are important therapeutic targets in FSGS, with implications for both slowing progression rate and improving renal survival.
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                Author and article information

                Contributors
                Journal
                Kidney Int Rep
                Kidney Int Rep
                Kidney International Reports
                Elsevier
                2468-0249
                20 March 2020
                June 2020
                20 March 2020
                : 5
                : 6
                : 860-871
                Affiliations
                [1 ]Division of Nephrology, Department of Medicine, Columbia University Medical Center, New York, New York, USA
                [2 ]Division of Nephrology, Spedali Civili and University of Brescia, Brescia, Italy
                [3 ]Division of Nephrology, Department of Pediatrics, North Carolina Children’s Hospital, Chapel Hill, North Carolina, USA
                [4 ]Division of Nephrology, Department of Medicine, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada
                [5 ]Renal-Electrolyte and Hypertension Division, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
                [6 ]Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA
                [7 ]Division of Nephrology, Virginia Commonwealth University, Richmond, Virginia, USA
                [8 ]Division of Nephrology, Maisonneuve-Rosemont Hospital, Department of Medicine, University of Montreal, Montreal, Quebec, Canada
                [9 ]Department of Medicine, Division of Renal Diseases and Hypertension, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina, USA
                [10 ]Division of Nephrology, Department of Pediatrics, University of Minnesota Masonic Children’s Hospital, Minneapolis, Minnesota, USA
                [11 ]Division of Nephrology, Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA
                [12 ]Division of Nephrology and Hypertension, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA
                [13 ]Division of Nephrology, Department of Medicine and Pediatrics, New York University Langone Health and New York University School of Medicine, New York, New York, USA
                [14 ]Renal Section, Texas Children’s Hospital, Baylor College of Medicine, Houston, Texas, USA
                Author notes
                [] Correspondence: Andrew S. Bomback, Columbia University Medical Center; 116th Street and Broadway, New York, New York 10027, USA. asb68@ 123456cumc.columbia.edu
                [15]

                Members of the CureGN Consortium are listed in the Appendix.

                Article
                S2468-0249(20)31125-6
                10.1016/j.ekir.2020.03.017
                7270998
                04aa33ec-43e7-41ef-8d00-2097f6560b2d
                © 2020 International Society of Nephrology. Published by Elsevier Inc.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 13 December 2019
                : 11 February 2020
                : 9 March 2020
                Categories
                Clinical Research

                focal segmental glomerulosclerosis,glomerular disease,glomerulonephropathy,iga nephropathy,membranous nephropathy,minimal change disease

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