Clinical Rheumatology

, Volume 37, Issue 4, pp 1065–1074 | Cite as

Determinants of renal and patient outcomes in a Spanish cohort of patients with ANCA-associated vasculitis and renal involvement

  • H. Marco
  • J. Draibe
  • J. Villacorta
  • L. F. Quintana
  • N. Martin
  • R. Garcia-Osuna
  • C. Cabre
  • MA. Martín-Gómez
  • A. Balius
  • A. Saurina
  • M. Picazo
  • I. Gich-Saladich
  • M. Navarro-Díaz
  • M. Praga
  • T. Cavero
  • J. Ballarin
  • M M Díaz-EncarnaciónEmail author
  • for the Spanish Group for the Study of Glomerular Disease (GLOSEN)
Original Article


The classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains controversial. The main objective of this study was to define the respective values of ANCA serotype-based classification, clinicopathological classification, and histopathological classification in predicting patient and renal outcomes in a Spanish cohort of patients with ANCA with specificity for myeloperoxidase, MPO-ANCA, versus ANCA with specificity for proteinase 3, PR3-ANCA. Two hundred and forty-five patients with ANCA-AAV and biopsy-proven renal involvement diagnosed between 2000 and 2104 were recruited in 12 nephrology services. Clinical and histologic data, renal outcomes, and mortality were analyzed. We applied the Chapel Hill Consensus Conference definition with categories for granulomatosis with the polyangiitis (GPA) and microscopic polyangiitis (MPA), the classification based on ANCA specificity, and the histopathological classification proposed in 2010. Eighty-two percent were MPO-ANCA positive and 18.0% PR3-ANCA positive. Altogether, 82.9% had MPA and 17.1% GPA. The median follow-up was 43.2 months (0.1–169.3). Neither ANCA-based serological nor clinical classification was predictive of renal outcomes or patient survival on bivariate or multivariate Cox regression analysis. Histopathological classification was found to predict development of end-stage renal disease (p = 0.005) in Kaplan-Meier analysis. ANCA specificity was more predictive of relapse than clinicopathological classification in multivariate analysis (HR 2.086; 95% CI 1.046–4.158; p = 0.037). In our Spanish cohort, a majority of patients had an MPO-ANCA-AAV. A classification based on ANCA specificity has a higher predictive value for relapse occurrence and could be used for decision-making with respect to induction treatment and maintenance therapies.


Clinicopathological classification Kidney biopsy MPO-ANCA PR3-ANCA Relapse Vasculitis 



We thank all the physicians who have been involved in the care of patients included in this study or have helped in the development of the study and this article.

Authors’ contributions

Jose Ballarin and Montserrat Diaz-Encarnación are joint last authors. They have contributed equally to the study.

Compliance with ethical standards

The study was compliance with ethical standards.




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Copyright information

© International League of Associations for Rheumatology (ILAR) 2018

Authors and Affiliations

  • H. Marco
    • 1
  • J. Draibe
    • 2
  • J. Villacorta
    • 3
  • L. F. Quintana
    • 4
  • N. Martin
    • 5
  • R. Garcia-Osuna
    • 6
  • C. Cabre
    • 7
  • MA. Martín-Gómez
    • 8
  • A. Balius
    • 9
  • A. Saurina
    • 10
  • M. Picazo
    • 11
  • I. Gich-Saladich
    • 12
  • M. Navarro-Díaz
    • 1
  • M. Praga
    • 13
  • T. Cavero
    • 13
  • J. Ballarin
    • 14
  • M M Díaz-Encarnación
    • 14
    Email author
  • for the Spanish Group for the Study of Glomerular Disease (GLOSEN)
  1. 1.Nephrology Division, Hospital Universitari Germans Trias i PujolUniversitat Autònoma de BarcelonaBadalonaSpain
  2. 2.Nephrology Division, Hospital Universitari de Bellvitge, BarcelonaUniversitat Autonoma de BarcelonaBarcelonaSpain
  3. 3.Nephrology DivisionHospital Universitario Fundación de AlcorcónMadridSpain
  4. 4.Nephrology DivisionHospital Universitari Clínic de BarcelonaBarcelonaSpain
  5. 5.Nephrology DivisionHospital Universitari Dr. J. TruetaGironaSpain
  6. 6.Nephrology DivisionHospital de PalamósGironaSpain
  7. 7.Nephrology DivisionHospital Universitari Joan XXIIITarragonaSpain
  8. 8.Nephrology DivisionHospital de PonienteAlmeriaSpain
  9. 9.Nephrology DivisionFundación AlthaiaManresaSpain
  10. 10.Nephrology DivisionHospital de TerrassaTerrassaSpain
  11. 11.Nephrology DivisionHospital de FigueresGironaSpain
  12. 12.Clinical Epidemiology Unit, Hospital de la Santa Creu i Sant PauUniversitat Autònoma de BarcelonaBarcelonaSpain
  13. 13.Nephrology DivisionHospital Universitario 12 de OctubreMadridSpain
  14. 14.Nephrology DivisionHospital Universitari Fundació Puigvert, Universitat Autònoma de BarcelonaBarcelonaSpain

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