Skip to main content

Long-term follow-up characteristics of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) receiving chronic hemodialysis at a single center



The clinical characteristics and treatment of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) after initiating chronic hemodialysis remain unknown.


We retrospectively enrolled 11 adult patients with AAV receiving chronic hemodialysis in our hospital from 2000–2016. We collected data describing each patient's clinical findings and treatment before and after initiating hemodialysis. Patients with AAV with and without post-hemodialysis AAV relapse were compared statistically.


The average observation period was 6.8 ± 4.1 years, and the interval between diagnosis and initiating chronic hemodialysis was 1.9 ± 2.6 years. Before initiating chronic hemodialysis, five patients (45%) experienced 12 AAV relapses, with diagnoses made serologically or symptomatically. After initiating chronic hemodialysis, four patients experienced nine relapses, with no significant difference between the number of relapses and the number of patients experiencing relapse (p = 0.067 and 0.083, respectively). For patients' entire clinical courses before initiating chronic hemodialysis, the average steroid dose was 11.6 ± 6.9 g/y. Comparing before and after initiating chronic hemodialysis, the steroid dose decreased significantly to 3.3 ± 1.4 g/y after initiating chronic hemodialysis (p = 0.0012). Two of 11 patients died of serious infections after initiating chronic hemodialysis.


Our results showed that the number of relapses tended to be lower despite a significantly different lower amount of steroid after initiating hemodialysis compared with before initiating hemodialysis, and the burn-out phenomenon specific to uremic patients was inferred. We believe that early tapering of steroids should be considered to avoid death rather than focusing only on relapse.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5


  1. 1.

    Jennette J, Falk R, Bacon P, Basu N, Cid M, Ferrario F, et al. 2012 Revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum. 2013;65:1–11.

    Article  CAS  Google Scholar 

  2. 2.

    Jennette CJ, Falk RJ, Andrassy K, Bacon PA, Churg J, Gross WL, et al. Nomenclature of systemic vasculitides. Arthritis Rheum. 1994;37:187–92.

    Article  CAS  Google Scholar 

  3. 3.

    Lionaki S, Hogan SL, Jennette CE, Hu Y, Hamra JB, Jennette CJ, et al. The clinical course of ANCA small-vessel vasculitis on chronic dialysis. Kidney Int. 2009;76:644–51.

    Article  Google Scholar 

  4. 4.

    Fujimoto S, Uezono S, Hisanaga S, Fukudome K, Kobayashi S, Suzuki K, et al. Incidence of ANCA-associated primary renal vasculitis in the Miyazaki prefecture: the first population-based, retrospective, epidemiologic survey in Japan. Clin J Am Soc Nephrol. 2006;1:1016–22.

    Article  Google Scholar 

  5. 5.

    Furuta S, Chaudhry AN, Arimura Y, Dobashi H, Fujimoto S, Homma S, et al. Comparison of the phenotype and outcome of granulomatosis with polyangiitis between UK and Japanese cohorts. The Journal of Rheumatology. 2016;44:216–22.

    Article  Google Scholar 

  6. 6.

    Mohammad AJ, Segelmark M. A population-based study showing better renal prognosis for proteinase 3 antineutrophil cytoplasmic antibody (ANCA)-associated nephritis versus myeloperoxidase ANCA-associated nephritis. J Rheumatol. 2014;41:1366–73.

    Article  Google Scholar 

  7. 7.

    Robson J, Doll H, Suppiah R, Flossmann O, Harper L, Höglund P, et al. Damage in the anca-associated vasculitides: long-term data from the European Vasculitis Study group (EUVAS) therapeutic trials. Ann Rheumat Dis 2013;74:2013–203927.

    Article  Google Scholar 

  8. 8.

    Sugiyama K, Sada K, Kurosawa M, Wada J, Makino H. Current status of the treatment of microscopic polyangiitis and granulomatosis with polyangiitis in Japan. Clin Exp Nephrol. 2013;17:51–8.

    Article  Google Scholar 

  9. 9.

    Yates M, Watts R, Bajema I, Cid M, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis. 2016;75:1583.

    Article  CAS  Google Scholar 

  10. 10.

    Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, Cohen P, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014;371:1771–800.

    Article  CAS  Google Scholar 

  11. 11.

    Sada K, Yamamura M, Harigai M, Fujii T, Takasaki Y, Amano K, et al. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015;17:305.

    Article  CAS  Google Scholar 

  12. 12.

    Watts R, Lane S, Hanslik T, Hauser T, Hellmich B, Koldingsnes W, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis. 2007;66:222.

    Article  Google Scholar 

  13. 13.

    Moiseev S, Novikov P, Jayne D, Mukhin N. End-stage renal disease in ANCA-associated vasculitis. Nephrol Dial Transpl. 2016;32:gfw046.

  14. 14.

    Hasegawa M, Hattori K, Sugiyama S, Asada H, Yamashita H, Takahashi K, et al. A retrospective study on the outcomes of MPO-ANCA-associated vasculitis in dialysis-dependent patients. Mod Rheumatol. 2015;26:110–4.

    Article  Google Scholar 

  15. 15.

    Okano K, Yumura W, Nitta K, Uchida K, Ohnuki T, Kawashima A, et al. Analysis of lupus activity in end-stage renal disease treated by hemodialysis. Intern Med. 2001;40:598–602.

    Article  CAS  Google Scholar 

  16. 16.

    Haubitz M, Koch K, Brunkhorst R. Survival and vasculitis activity in patients with end-stage renal disease due to Wegener’s granulomatosis. Nephrol Dial Transplant. 1998;13:1713–8.

    Article  CAS  Google Scholar 

  17. 17.

    Memoli B, Marzano L, Bisesti V, Andreucci M, Guida B. Hemodialysis-related lymphomononuclear release of interleukin-12 in patients with end-stage renal disease. J Am Soc Nephrol JASN. 1999;10:2171–6.

    PubMed  CAS  Google Scholar 

  18. 18.

    Banche G, Allizond V, Giacchino F, Mandras N, Roana J, Bonello F, et al. Effect of dialysis membrane biocompatibility on polymorphonuclear granulocyte activity in dialysis patients. Nephrol Dial Transplant. 2006;21:3532–8.

    Article  CAS  Google Scholar 

  19. 19.

    Dinarello CA. Cytokines: Agents provocateurs in hemodialysis? Kidney Int. 1992;41:683–94.

    Article  CAS  Google Scholar 

  20. 20.

    Chonchol M. HEMATOLOGY: ISSUES IN THE DIALYSIS PATIENT: neutrophil dysfunction and infection risk in end-stage renal disease. Semin Dial. 2006;19:291–6.

    Article  Google Scholar 

  21. 21.

    Vanholder R, Ringoir S. Infectious morbidity and defects of phagocytic function in end-stage renal disease: a review. J Am Soc Nephrol JASN. 1993;3:1541–54.

    PubMed  CAS  Google Scholar 

  22. 22.

    Jennette CJ, Falk RJ. Pathogenesis of antineutrophil cytoplasmic autoantibody-mediated disease. Nat Rev Rheumatol. 2014;10:nrrheum.2014.103.

    Article  CAS  Google Scholar 

  23. 23.

    Kemna MJ, Damoiseaux J, Austen J, Winkens B, Peters J, van Paassen P, et al. ANCA as a predictor of relapse: useful in patients with renal involvement but not in patients with nonrenal disease. J Am Soc Nephrol. 2015;26:537–42.

    Article  CAS  Google Scholar 

  24. 24.

    Romeu M, Couchoud C, Delarozière J-C, Burtey S, Chiche L, Harlé J-R, et al. Survival of patients with ANCA-associated vasculitis on chronic dialysis: data from the French REIN registry from 2002 to 2011. QJM. 2014;107:545–55.

    Article  CAS  Google Scholar 

  25. 25.

    Weidanz F, Day CJ, Hewins P, Savage CO, Harper L. Recurrences and infections during continuous immunosuppressive therapy after beginning dialysis in ANCA-associated vasculitis. Am J Kidney Dis. 2007;50:36–46.

    Article  Google Scholar 

  26. 26.

    McGregor JG, Hogan SL, Hu Y, Jennette CE, Falk RJ, Nachman PH. Glucocorticoids and relapse and infection rates in anti-neutrophil cytoplasmic antibody disease. Clin J Am Soc Nephrol. 2012;7:240–7.

    Article  CAS  Google Scholar 

  27. 27.

    Wada T, Hara A, Arimura Y, Sada K-E, Makino H, and of of of Vasculitis W. Risk factors associated with relapse in Japanese patients with microscopic polyangiitis. J Rheumatol 2011;39:545–51.

    Article  CAS  Google Scholar 

  28. 28.

    Walsh M, Flossmann O, Berden A, Westman K, Höglund P, Stegeman C, et al. Risk factors for relapse of antineutrophil cytoplasmic antibody–associated vasculitis. Arthritis Rheum. 2012;64:542–8.

    Article  CAS  Google Scholar 

  29. 29.

    Walsh M, Merkel PA, Mahr A, Jayne D. Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: a meta-analysis. Arthritis Care Res. 2010;62:1166–73.

    Article  Google Scholar 

  30. 30.

    de Jager DJ, Grootendorst DC, Jager KJ, van Dijk PC, Tomas LM, Ansell D, et al. Cardiovascular and noncardiovascular mortality among patients starting dialysis. JAMA. 2009;302:1782–9.

    Article  Google Scholar 

  31. 31.

    Tentori F, McCullough K, Kilpatrick RD, Bradbury BD, Robinson BM, Kerr PG, et al. High rates of death and hospitalization follow bone fracture among hemodialysis patients. Kidney Int. 2014;85:166–73.

    Article  Google Scholar 

Download references


We thank Jane Charbonneau, DVM, from Edanz Group ( for editing a draft of this manuscript.



Author information



Corresponding author

Correspondence to Kazunori Karasawa.

Ethics declarations

Conflict of interest

The authors have declared that no conflict of interest exists.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee at which the studies were conducted (IRB approval number 4370) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

About this article

Verify currency and authenticity via CrossMark

Cite this article

Miyabe, Y., Karasawa, K., Takabe, T. et al. Long-term follow-up characteristics of patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) receiving chronic hemodialysis at a single center. Clin Exp Nephrol 24, 136–142 (2020).

Download citation


  • AAV
  • Hemodialysis
  • Burn-out phenomenon