AN UPDATE ON GLOMERULOPATHIES – CLINICAL AND TREATMENT ASPECTS Edited by Sharma S. Prabhakar An Update on Glomerulopathies – Clinical and Treatment Aspects Edited by Sharma S. Prabhakar Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia Copyright © 2011 InTech All chapters are Open Access distributed under the Creative Commons Attribution 3.0 license, which permits to copy, distribute, transmit, and adapt the work in any medium, so long as the original work is properly cited. After this work has been published by InTech, authors have the right to republish it, in whole or part, in any publication of which they are the author, and to make other personal use of the work. Any republication, referencing or personal use of the work must explicitly identify the original source. 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Used under license from Shutterstock.com First published October, 2011 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechweb.org An Update on Glomerulopathies – Clinical and Treatment Aspects, Edited by Sharma S. Prabhakar p. cm. ISBN 978-953-307-673-7 free online editions of InTech Books and Journals can be found at www.intechopen.com Contents Foreword IX Preface XI Part 1 Primary Glomerulopathies 1 Chapter 1 Membranous Nephropathy 3 Abdelaziz Elsanjak and Sharma S. Prabhakar Chapter 2 Focal Segmental Glomerulosclerosis 25 Dawinder S. Sohal and Sharma S Prabhakar Chapter 3 Membranoproliferative Glomerulonephritis 45 Matthew C. Pickering and Joshua M. Thurman Chapter 4 Primary IgA Nephropathy: An Update in 2011 59 Francois Berthoux and Amir Kamal Aziz Chapter 5 IgA Nephropathy: Insights into Genetic Basis and Treatment Options 69 Dimitrios Kirmizis, Aikaterini Papagianni and Francesco Paolo Schena Chapter 6 Rapidly Progressive Glomerulonephritis 95 Maria Pia Rastaldi Part 2 Infectious Glomerulopathies and Related Disorders 111 Chapter 7 Post-Infectious Glomerulonephritis 113 Gurmeet Singh Chapter 8 S. pyogenes Infections and Its Sequelae 125 L. Guilherme, S. Freschi de Barros A.C. Tanaka, M.C. Ribeiro Castro and J. Kalil Chapter 9 Insights from Genomics on Post-Infectious Streptococcal Glomerulonephritis 137 W. Michael McShan and Roya Toloui VI Contents Chapter 10 Atypical Clinical Manifestations of Acute Poststreptococcal Glomerulonephritis 151 Toru Watanabe Chapter 11 Hepatitis C Virus Associated Glomerulonephritis 169 Vincent Ho and Jason Chen Chapter 12 Glomerular Pathology in Patients with HIV Infection 189 Enrique Morales, Elena Gutierrez-Solis, Eduardo Gutierrez and Manuel Praga Part 3 Vasculitis and Autoimmune Glomerulopathies 207 Chapter 13 Henoch-Schönlein Purpura Nephritis in Childhood 209 Marco Zaffanello Chapter 14 Lupus Glomerulonephritis 231 Chi Chiu Mok Chapter 15 Anti-Glomerular Basement Membrane Disease 251 Kouichi Hirayama and Kunihiro Yamagata Chapter 16 Mixed Hematopoietic Chimerism Allows Cure of Autoimmune Glomerulonephritis: Its Potential and Risks 277 Emiko Takeuchi Chapter 17 Differential Diagnosis of the Pulmonary-Renal Syndrome 291 Martin Kimmel, Niko Braun and Mark Dominik Alscher Chapter 18 RPGN - Clinical Features, Treatment and Prognosis 299 Mitra Naseri Part 4 Glomerular Disease in Metabolic and Systemic Conditions 325 Chapter 19 Diabetic Glomerulopathy 327 Mahmoud Barazi, Harneet Kaur and Sharma Prabhakar Chapter 20 Metabolic Syndrome Associated Kidney Damage 369 Hequn Zou, Yuxin Wang, Guimian Zou and Jianxin Wan Chapter 21 Glomerulonephritis and the Cystic Fibrosis Patient 399 Daniel Fischman, Arvin Parvathaneni and Pramil Cheriyath Contents VII Chapter 22 Mild Forms of Alport Syndrome: Hereditary Nephropathy in the Absence of Extra-Renal Features 417 Han-Seung Yoon and Michael R. Eccles Part 5 Miscellaneous Topics 437 Chapter 23 Nephrotic Syndrome in Children – Studies from South Africa 439 Gertruida van Biljon Chapter 24 Blood Pressure Control in Patients with Glomerulonephritis 455 Toshihiko Ishimitsu Foreword The study of the fundamental and clinical aspects of glomerular disease has expanded exponentially over the last several decades. Fresh insights into old clinico-pathological entities emerge frequently and continuously. New disease entities also arise as the sophistication of investigation expands. Novel disease-specific treatments are applied often with impressive results while older, more empiric therapeutic strategies are in a constant state of evaluation and re-evaluation. Thus a book dealing with recent advances in the field of glomerulopathies from both clinical and therapeutic aspects is a welcome addition to our knowledge base. An Update on Glomerulopathies - Clinical and Treatment Aspects builds on the background provided by An Update on Glomerulopathies - Etiology and Pathogenesis. In 24 concise, focused and well-formulated chapters the broad scope of clinical and therapeutic aspects of primary and secondary glomerular disease is reviewed and updated. The Editor, Dr. Sharma Prabhakar has chosen both the topics and the authors wisely. The contributions will have great appeal and value to the practicing clinician who frequently must face diagnostic and management challenges in this arena of medicine. While not exhaustive in coverage, the topics included embrace an impressive breadth of clinical experience in these disorders. The contributions also examine contemporary issues in sufficient detail to serve as an excellent guide for the clinician as well as trainees in nephrology. An Update on Glomerulopathies - Clinical and Treatment Aspects illustrates how far we have come in our understanding of clinical issues in glomerular disease, but also shows how much more needs to be understood. Richard J. Glassock, MD, MACP Laguna Niguel, California USA [...]... basement membrane (GBM), caused by subepithelial immune complex deposits The immune 4 An Update on Glomerulopathies – Clinical and Treatment Aspects deposits consist of IgG, mainly IgG4 and IgG1 of antigens that have long escaped identification, and the membrane attack complex of complement C5b-9 (MAC) The formation of subepithelial immune deposits and complement activation are responsible for functional impairment... predominantly in their 30s and 40s, and has an enormous long-term impact on their quality of life and productivity Because they have single-organ disease rather than multisystem organ failure (as is seen in diabetes), they survive longer on dialysis and after renal transplantation However, even though these patients survive longer, they continue to function at a lower level in comparison with the age and. .. experiencing spontaneous remission This wide variation in outcome is one of the factors that has led meta-analysis and systematic reviews of this disease to reach varying conclusions about the impact of immunosuppressive treatment on patient and renal survival and on proteinuria remission rates Female gender and low grade proteinuria is associated with good prognosis and associated with spontaneous remission End-stage... despite 6 months of conservative therapy," Ten patients achieved a full remission after treatment; however, they were more likely female and with lower baseline serum creatinine values, which is a population of high spontaneous remission Recently, a small RCT study 18 An Update on Glomerulopathies – Clinical and Treatment Aspects conducted in Spain demonstrated that rituximab was of benefit in 13 Spanish... history and prognosis of idiopathic MN MN is a chronic disease, with spontaneous remission and relapses In the United States and Europe, MN remains the second or third leading cause of ESRD among the primary glomerulonephritis types Spontaneous remissions occur in up to 30% of cases and usually occur within the first 2 yrs after presentation The percentage of patients going into spontaneous remission is... immunosuppressive agents and newer, potentially less toxic agents (eg, mycophenolate or rituximab) have been introduced for the treatment of MN without the benefit of long-term clinical trials A meta-analysis on 1025 patients with MN from 18 randomized clinical trials concluded that immunosuppressive treatment had no 14 An Update on Glomerulopathies – Clinical and Treatment Aspects benefits in patient... described Preface in a concise and clear manner the significance of and approaches to blood pressure control in the context of glomerular disease In summary, An Update on Glomerulopathies - Clinical and Treatment Aspects is a systemic overview of recent advances in clinical aspects and therapeutic options in major syndromes of glomerular pathology While by no means this book replaces currently available... membrane In context of diseases associated with the nephrotic syndrome, the differential diagnosis includes minimal change disease, focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis, diabetes mellitus and amyloidosis In the past, before the development of electron microscopy and immunologic 12 An Update on Glomerulopathies – Clinical and Treatment Aspects techniques, many... falls to 90% with partial remission, and 45% with no remission Cattran and his colleagues (Cattran 2005) proposed a prognostic model dividing patients with into low-, moderate-, and highrisk groups based on their degree of proteinuria and clinical course over 6 months of observation Those with normal renal function and lower amounts of proteinuria . AN UPDATE ON GLOMERULOPATHIES – CLINICAL AND TREATMENT ASPECTS Edited by Sharma S. Prabhakar An Update on Glomerulopathies – Clinical and Treatment Aspects. base. An Update on Glomerulopathies - Clinical and Treatment Aspects builds on the background provided by An Update on Glomerulopathies - Etiology and Pathogenesis. In 24 concise, focused and. immune An Update on Glomerulopathies – Clinical and Treatment Aspects 4 deposits consist of IgG, mainly IgG4 and IgG1 of antigens that have long escaped identification, and the membrane attack