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      Vasculitic IgA Nephropathy: Prognosis and Outcome

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          Abstract

          Background: A vasculitic glomerulonephritis is seen in both IgA nephropathy and Henoch-Schönlein purpura. No study has examined prognostic factors in vasculitic IgA nephropathy. Methods: All patients with vasculitic IgA presenting to our centre between January 1996 and May 2003 were retrospectively reviewed by analysis of clinical and pathological features at presentation and during follow-up. Results: Of 363 patients with IgA nephropathy, 67 patients had vasculitic IgA nephropathy (33 Henoch-Schönlein purpura; 34 IgA). Median length of follow-up was 32.38 months (range 0–90). Median glomerular filtration rate at presentation was 45.56 ml/min (range 4–110), arterial blood pressure 104.67 mm Hg and proteinuria 1.19 g/24 h (range 0–14.8) falling during follow-up to 0.11 g (range 0–3.1). Median chronic damage at presentation in the biopsy was 10% (range 0–91). The majority (79%) were immunosuppressed with 84% patient survival and 85% renal survival at 60 months. Three factors significantly affected renal outcome: renal function; blood pressure at presentation and the amount of chronic damage in the biopsy. The effect of immunosuppression on outcome was difficult to comment on as treated and untreated groups were not comparable. Conclusions: Presenting renal function, blood pressure and chronic damage in the biopsy are important prognostic factors in vasculitic IgA nephropathy. Immunosuppression is advocated in some patients.

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          Henoch-Schönlein Purpura in adults: outcome and prognostic factors.

          Henoch-Schönlein Purpura nephritis (HSPN) has been extensively studied in children but, its natural history in adults is much less known. A cohort of 250 adults suffering HSP was retrospectively analyzed for a median follow-up period of 14.8 yr. All patients had biopsies consistent with HSP (predominant IgA mesangial deposits) associated with purpura, bowel angina, and/or abdominal pain. At presentation, palpable purpura was present in 96% of patients, and arthritis was reported in 61%, and gastrointestinal involvement in 48%. Thirty-two percent of the patients showed renal insufficiency (Creatinine clearance [CrCl] <50 ml/min), usually associated with proteinuria (99%) and/or hematuria (93%). Endocapillary glomerulonephritis was the most frequent lesion on renal biopsy (61%). At the end of follow-up, patient survival was only 74%. The first cause of death was carcinoma (most of them of respiratory or digestive tract). Regarding renal function, 11% of patients reached end-stage renal failure, 13% exhibited severe renal failure (CrCl <30 ml/min), and 14% moderate renal insufficiency (CrCl <50 ml/min). Clinical remission defined as the absence of proteinuria, hematuria, and a normal renal function was achieved in only 20%. This is a retrospective study; therefore, it is not possible to demonstrate any steroid and/or cyclophosphamide efficacy in diminishing the incidence of renal insufficiency. Multivariate analysis demonstrated that renal function impairment and proteinuria level at presentation and, on renal biopsy, the degree of interstitial fibrosis, percentage of sclerotic glomeruli, and presence of glomeruli with fibrinoid necrosis were associated with a poor renal prognosis. The data indicate that clinical presentation of HSPN in adults is severe and its outcome relatively poor, worse than in children. Identification of clinical and histologic prognostic factors may permit the design of appropriate therapeutic prospective studies.
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            Predicting progression in IgA nephropathy.

            Immunoglobulin A (IgA) nephropathy is one of the most common primary types of glomerulonephritis to progress to end-stage renal disease. Its variable and often long natural history makes it difficult to predict outcome. We investigated the association of the rate of renal function decline based on the slope of creatinine clearance over time with demographic, clinical, laboratory, and histological data from 298 patients with biopsy-proven IgA nephropathy with a mean follow-up of 70 months. Using univariate analysis, urinary protein excretion at baseline and Lee pathological grading, as well as mean arterial pressure (MAP) and urinary protein excretion during follow-up, were associated with the rate of deterioration in renal function. Of these, only MAP and urinary protein excretion during follow-up were identified as independent factors by multiple linear regression analysis. The combination of best accuracy of prediction and shortest observation time using these two parameters was reached between the second and third years of follow-up. A semiquantitative method of estimating the rate of progression by using these factors was developed. These results indicate that MAP and severity of proteinuria over time are the most important prognostic indicators of IgA nephropathy. The potential relevance of the algorithm in patient management is shown.
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              A tricontinental view of IgA nephropathy.

              The purpose of this retrospective study was to analyse patients from four centres in three continents to determine if differences in long-term outcome of IgA nephropathy (IgAN) are explained by clinical and laboratory features at presentation. The study included 711 adults with biopsy-proven IgAN from Glasgow, UK (n = 112), Helsinki, Finland (n = 204), Sydney, Australia (n = 121) and Toronto, Canada (n = 274). Data collected from time of presentation to a nephrologist were age, gender, 24-h urine protein excretion (UP(0)), mean arterial pressure (MAP(0)) and creatinine clearance (CrCl(0)). Outcomes were slope of creatinine clearance (CrCl) and renal survival. At presentation there was significant vari-ability in baseline clinical features with patients from Helsinki having the lowest median UP(0), lowest MAP(0) and highest CrCl(0), all suggesting milder disease. There was significant variability in renal survival between centres with 10-year actuarial survival of 95.7, 87.0, 63.9 and 61.6% in Helsinki, Sydney, Glasgow and Toronto, respectively (P < 0.0001; log rank). Cox proportional hazards model revealed lower age(0) and lower CrCl(0) were significant independent predictors of reduced renal survival. In addition, patients from Helsinki and Sydney but not Glasgow had significantly longer renal survival than patients from Toronto. Median slope of CrCl varied by region from -1.24 ml/min/year in Helsinki, to -3.99 ml/min/year in Toronto (Kruskal-Wallis H test P < 0.0001). By multivariate analysis older age(0), higher CrCl(0) and lower UP(0) were independently associated with slower progression. Subjects from Helsinki had a significantly slower deterioration independent of the other clinical parameters at presentation. When the 269 patients presenting with CrCl(0) <75 ml/min were analysed separately there was no independent centre effect. The findings are consistent with the hypothesis that geographical variability in long-term outcome of IgAN is explained by lead-time bias and inclusion of milder cases in centres with apparent good outcome, but do not exclude the possibility that some of the variability is due to other factors such as genetics, diet or treatment.
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                Author and article information

                Journal
                NEC
                Nephron Clin Pract
                10.1159/issn.1660-2110
                Nephron Clinical Practice
                S. Karger AG
                1660-2110
                2009
                May 2009
                03 April 2009
                : 112
                : 1
                : c16-c24
                Affiliations
                aDepartment of Immunity and Infection, University of Birmingham, bUniversity Hospitals Trust, Birmingham, and cDepartment of Pathology, University College London, London, UK; dDepartment of Nephrology and Intensive Care, Charité Campus Virchow, University Hospital, Berlin, Germany
                Article
                210570 Nephron Clin Pract 2009;112:c16–c24
                10.1159/000210570
                19342865
                cdd0527f-0089-4f90-8d5b-56e086e9db74
                © 2009 S. Karger AG, Basel

                Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                History
                : 14 August 2008
                : 03 October 2008
                Page count
                Figures: 2, Tables: 5, References: 39, Pages: 1
                Categories
                Original Paper

                Cardiovascular Medicine,Nephrology
                Vasculitic IgA nephropathy,Chronic kidney disease,Henoch-Schönlein purpura,Crescentic lesions,Glomerulonephritis,IgA nephropathy

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