49. End-Stage Renal Disease and Vasculitis

  1. Gary S. Hoffman MD, MS3,
  2. Cornelia M. Weyand MD, PhD4,
  3. Carol A. Langford MD, MHS3 and
  4. Jörg J. Goronzy MD, PhD4
  1. Kirsten de Groot MD1 and
  2. Charles Pusey DSc, FRCP2

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch49

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

de Groot, K. and Pusey, C. (2012) End-Stage Renal Disease and Vasculitis, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch49

Editor Information

  1. 3

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 4

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. 1

    3rd Medical Department, Section of Nephrology and Rheumatology, Klinikum Offenbach, GmbH, KfH Nierenzentrum Offenbach, Offenbach/Main, Germany

  2. 2

    Renal Section, Department of Medicine, Imperial College London, London, UK

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Small vessel vasculitis;
  • ANCA;
  • necrotizing crescentic glomerulonephritis;
  • rapidly progressive glomerulonephritis;
  • immunosuppression;
  • plasma exchange;
  • maintenance hemodialysis;
  • kidney transplantation;
  • predictors of independent renal function


Among the vasculitides with renal involvement, the course and outcome of renal disease has been most fully studied in ANCA-associated vasculitides (AAV). End-stage renal disease (ESRD) can result from rapidly progressive glomerulonephritis (RPGN), chronic smouldering renal disease or continued scarring. The aim of inducing remission and restoring dialysis independent renal function in acute disease is pursued by immunosuppressive therapy, including cyclophosphamide and corticosteroids in combination with plasma exchange. However, within 5 years 25% of patients presenting with RPGN will develop ESRD. In this situation, either hemodialysis or peritoneal dialysis can be used for renal replacement therapy. Mortality in these patients is higher than in AAV patients maintaining independent renal function, but is similar to other cohorts of dialysis patients. Once remission is achieved, renal transplantation is a further option, offering results similar to other causes of ESRD. The frequency of disease relapse is comparable between patients with and without renal failure in most studies, whereas it is significantly lower after renal transplantation.