16
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      IgA nephropathy in Brazil: apropos of 600 cases

      research-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          IgA nephropathy (IgAN) is th e commonest primary glomerular disease worldwide. Studies on its prevalence in Brazil are however scarce. Databases and clinical records from 10 reference centres were retrospectively reviewed. Clinical and laboratory features at the moment of the biopsy were retrieved (age, gender, presence of hematuria, serum creatinine [mg/dL], proteinuria [g/24 h]). Renal biopsy findings were classified according to Haas single grade classification scheme and the Oxford Classification of IgAN. 600 cases of IgAN were identified, of which 568 (94.7 %) were on native kidneys. Male to female ratio was 1.24:1. Patients averaged 32.76 ± 15.12 years old (range 4–89, median 32). Proteinuria and hematuria were observed, respectively in 56.63 and 72.29 % of patients. The association of both these findings occurred in 37.95 % of the cases. Serum creatinine averaged 1.65 ± 0.67 mg/dL (median 1.5 mg/dL) at diagnosis. Segmental sclerosis and mesangial hypercellularity were the main glomerular findings (47.6 and 46.2 %) The commonest combination by Oxford Classification of IgAN, was M0 E0 S0 T0 (22.4 %). Chronic tubulo-interstitial lesions with an extension wider than 25 % of the renal cortex could be identified in 32.2 % of the cases. Tubular atrophy and interstitial fibrosis were more strongly associated with higher 24-h proteinuria and serum creatinine levels. Segmental sclerosis (S1) showed a stronger tendency of association with the presence of tubulo-interstitial lesions (T1 and T2) than other glomerular variables. To the best of our knowledge this is the largest series of IgAN in Brazil. It depicts the main biopsy findings and their possible clinical correlates. Our set of data is comparable to previous reports.

          Related collections

          Most cited references24

          • Record: found
          • Abstract: found
          • Article: not found

          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.
            Bookmark
            • Record: found
            • Abstract: found
            • Article: found
            Is Open Access

            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.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Pathogenesis of IgA nephropathy.

              Since its first description in 1968, IgA nephropathy has remained the most common form of idiopathic glomerulonephritis leading to chronic kidney disease in developed countries. The exact pathogenesis of IgA nephropathy is still not well defined. Current data implicate an important genetic factor, especially in promoting the overproduction of an aberrant form of IgA1. The immunochemical aberrancy of IgA nephropathy is characterized by the undergalactosylation of O-glycans in the hinge region of IgA1. However, such aberrant glycosylation alone does not cause renal injury. The next stage of disease development requires the formation of glycan-specific IgG and IgA antibodies that recognize the undergalactosylated IgA1 molecule. These antibodies often have reactivity against antigens from extrinsic microorganisms and might arise from recurrent mucosal infection. B cells that respond to mucosal infections, particularly tonsillitis, might produce the nephritogenic IgA1 molecule. With increased immune-complex formation and decreased clearance owing to reduced uptake by the liver, IgA1 binds to the glomerular mesangium via an as yet unidentified receptor. Glomerular IgA1 deposits trigger the local production of cytokines and growth factors, leading to the activation of mesangial cells and the complement system. Emerging data suggest that mesangial-derived mediators following glomerular deposition of IgA1 lead to podocyte and tubulointerstitial injury via mesangio-podocytic-tubular crosstalk. This Review summarizes the latest findings in the pathogenesis of IgA nephropathy.
                Bookmark

                Author and article information

                Contributors
                +55 41 3260-7240 , mafer.soares@gmail.com , mafer.soares@ufpr.br
                Journal
                Springerplus
                Springerplus
                SpringerPlus
                Springer International Publishing (Cham )
                2193-1801
                24 September 2015
                24 September 2015
                2015
                : 4
                : 547
                Affiliations
                [ ]Department of Pathology, Federal University of São Paulo (UNIFESP), São Paulo, Brazil
                [ ]Department of Medical Pathology, Federal University of Paraná (UFPR), Curitiba, Brazil
                [ ]Department of Pathology, Federal Fluminense University (UFF), Niterói, Brazil
                [ ]Centro de Pesquisas Gonçalo Moniz, Oswaldo Cruz Foundation (CPqGM-FIOCRUZ), Salvador, Brazil
                [ ]Santos Metropolitan University (UNIMES) and Ana Costa Hospital, Santos, Brazil
                [ ]Clinics Hospital, Federal University of Minas Gerais (UFMG), Belo Horizonte, Brazil
                [ ]Federal University of Ouro Preto (UFOP), Ouro Prêto, Brazil
                [ ]Santa Casa de Misericórdia de Porto Alegre, Porto Alegre, Brazil
                [ ]Brazilian Kidney Club, Brazilian Society of Pathology (SBP), São Paulo, Brazil
                [ ]Division of Health Sciences, Federal University of Parana (UFPR), Curitiba, Brazil
                [ ]Departamento de Patologia Médica, Rua Padre Camargo, 280, 6. andar, Curitiba, PR 80060-240 Brazil
                Article
                1323
                10.1186/s40064-015-1323-x
                4582041
                2ed2eea8-f21d-4be4-8e32-d7b3db98d9e6
                © Soares et al. 2015

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

                History
                : 29 June 2015
                : 8 September 2015
                Categories
                Research
                Custom metadata
                © The Author(s) 2015

                Uncategorized
                iga nephropathy,berger’s disease,pathology,epidemiology
                Uncategorized
                iga nephropathy, berger’s disease, pathology, epidemiology

                Comments

                Comment on this article