Advertisement

The complexity of classifying ANCA-associated small-vessel vasculitis in actual clinical practice: data from a multicenter retrospective survey

Abstract

The different sets of criteria for diagnosis or classification of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) lead to numerous overlapping and reclassified diagnoses in clinical practice. We designed this study to assess the difficulties in classifying patients with AAV. As a secondary objective, different variables were tested to predict prognosis. We conducted a retrospective chart review in a Western Spain multicentre survey. A total of 115 adult patients diagnosed with AAV from 2002 to 2013 and followed for at least 3 years were included. They were classified according to (1) Chapel Hill Consensus Conference (CHCC), (2) European Medicines Agency algorithm and (3) French Vasculitis Study Group/European Vasculitis Society phenotypes. Fifty-three patients (46%) had neither distinctive histopathological data of a single AAV definition nor any surrogate markers for granulomatous inflammation and thus did not fulfill any diagnostic criteria. Ocular, ear, nose, throat, skin, and lung involvement were more frequent with proteinase 3 (PR3) antibodies, whereas peripheral neuropathy was more frequent with myeloperoxidase (MPO) antibodies. When the disease was severe at diagnosis, the HR for mortality was 10.44. When induction treatment was not given in accordance with the guidelines, the HR for mortality was 4.00. For maintenance treatment, the HR was 5.49 for mortality and 2.48 for relapse. AAV classification is difficult because many patients had neither specific clinical data nor distinctive histological features of a single CHCC definition. A structured clinical assessment of patient severity is the best tool to guide the management of AAV.

This is a preview of subscription content, log in to check access.

Access options

Buy single article

Instant unlimited access to the full article PDF.

US$ 39.95

Price includes VAT for USA

Subscribe to journal

Immediate online access to all issues from 2019. Subscription will auto renew annually.

US$ 199

This is the net price. Taxes to be calculated in checkout.

Fig. 1

References

  1. 1.

    Binda V, Moroni G, Messa P (2018) ANCA-associated vasculitis with renal involvement. J Nephrol 31:197–208. https://doi.org/10.1007/s40620-017-0412-z

  2. 2.

    Leavitt RY, Fauci AS, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107

  3. 3.

    Masi AT, Hunder GG, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33:1094–1100

  4. 4.

    Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 37:187–192

  5. 5.

    Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised international chapel hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11. https://doi.org/10.1002/art.37715

  6. 6.

    Sørensen SF, Slot O, Tvede N, Petersen J (2000) A prospective study of vasculitis patients collected in a five year period: evaluation of the Chapel Hill nomenclature. Ann Rheum Dis 59:478–482

  7. 7.

    Watts R, Lane S, Hanslik T et al (2007) Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 66:222–227. https://doi.org/10.1136/ard.2006.054593

  8. 8.

    Yoo J, Kim HJ, Ahn SS et al (2018) The utility of the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis in Korean patients with antineutrophil cytoplasmic antibody-associated vasculitis. Clin Exp Rheumatol 36(Suppl 111):85–87

  9. 9.

    Basu N, Watts R, Bajema I et al (2010) EULAR points to consider in the development of classification and diagnostic criteria in systemic vasculitis. Ann Rheum Dis 69:1744–1750. https://doi.org/10.1136/ard.2009.119032

  10. 10.

    Bruce IN, Bell AL (1997) A comparison of two nomenclature systems for primary systemic vasculitis. Br J Rheumatol 36:453–458

  11. 11.

    Lane SE, Watts RA, Shepstone L, Scott DGI (2005) Primary systemic vasculitis: clinical features and mortality. QJM 98:97–111. https://doi.org/10.1093/qjmed/hci015

  12. 12.

    Lionaki S, Blyth ER, Hogan SL et al (2012) Classification of antineutrophil cytoplasmic autoantibody vasculitides: the role of antineutrophil cytoplasmic autoantibody specificity for myeloperoxidase or proteinase 3 in disease recognition and prognosis. Arthritis Rheum 64:3452–3462. https://doi.org/10.1002/art.34562

  13. 13.

    Lane SE, Watts RA, Barker THW, Scott DGI (2002) Evaluation of the Sørensen diagnostic criteria in the classification of systemic vasculitis. Rheumatology (Oxford) 41:1138–1141

  14. 14.

    Mahr A, Katsahian S, Varet H et al (2013) Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody-associated vasculitis: a cluster analysis. Ann Rheum Dis 72:1003–1010. https://doi.org/10.1136/annrheumdis-2012-201750

  15. 15.

    Corral-Gudino L et al (2013) Antineutrophil cytoplasmic autoantibodies: an unbiased and efficient tool to classify renal vasculitis? Comment on the article by Lionaki et al. Arthritis Rheum 65:1405–1406. https://doi.org/10.1002/art.37892

  16. 16.

    Miloslavsky EM, Lu N, Unizony S et al (2016) Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-positive and ANCA-negative patients with granulomatosis with polyangiitis (Wegener’s): distinct patient subsets. Arthritis Rheumatol (Hoboken, NJ) 68:2945–2952. https://doi.org/10.1002/art.39812

  17. 17.

    Schirmer JH, Wright MN, Herrmann K et al (2016) Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-positive granulomatosis with polyangiitis (Wegener’s) is a clinically distinct subset of ANCA-associated vasculitis: a retrospective analysis of 315 patients from a German vasculitis referral center. Arthritis Rheumatol (Hoboken, NJ) 68:2953–2963. https://doi.org/10.1002/art.39786

  18. 18.

    Solans-Laqué R, Fraile G, Rodriguez-Carballeira M et al (2017) Clinical characteristics and outcome of Spanish patients with ANCA-associated vasculitides: impact of the vasculitis type, ANCA specificity, and treatment on mortality and morbidity. Medicine (Baltimore) 96:e6083. https://doi.org/10.1097/MD.0000000000006083

  19. 19.

    Deshayes S, Martin Silva N, Khoy K et al (2019) Clinical impact of subgrouping ANCA-associated vasculitis according to antibody specificity beyond the clinicopathological classification. Rheumatology (Oxford). https://doi.org/10.1093/rheumatology/kez016

  20. 20.

    Vassar M, Holzmann M (2013) The retrospective chart review: important methodological considerations. J Educ Eval Health Prof 10:12. https://doi.org/10.3352/jeehp.2013.10.12

  21. 21.

    Mukhtyar C, Lee R, Brown D et al (2009) Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Ann Rheum Dis 68:1827–1832. https://doi.org/10.1136/ard.2008.101279

  22. 22.

    Levey AS, Stevens LA, Schmid CH et al (2009) A new equation to estimate glomerular filtration rate. Ann Intern Med 150:604–612

  23. 23.

    Mukhtyar C, Guillevin L, Cid MC et al (2009) EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 68:310–317. https://doi.org/10.1136/ard.2008.088096

  24. 24.

    Yates M, Watts RA, Bajema IM et al (2016) EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 75:1583–1594. https://doi.org/10.1136/annrheumdis-2016-209133

  25. 25.

    Exley AR, Bacon PA, Luqmani RA et al (1997) Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum 40:371–380

  26. 26.

    Lanham JG, Elkon KB, Pusey CD, Hughes GR (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome. Medicine (Baltimore) 63:65–81

  27. 27.

    Gatenby PA, Lucas RM, Engelsen O et al (2009) Antineutrophil cytoplasmic antibody-associated vasculitides: could geographic patterns be explained by ambient ultraviolet radiation? Arthritis Rheum 61:1417–1424. https://doi.org/10.1002/art.24790

  28. 28.

    Corral-Gudino L, Borao-Cengotita-Bengoa M, Lerma-Marquez JL, del Pino-Montes J (2011) Differences in the incidence of microscopic polyangiitis and granulomatosis with polyangiitis (Wegener’s). Is there a latitudinal gradient? J Rheumatol 38:2494–2496. https://doi.org/10.3899/jrheum.110650

  29. 29.

    Luqmani RA, Suppiah R, Grayson PC et al (2011) Nomenclature and classification of vasculitis—update on the ACR/EULAR diagnosis and classification of vasculitis study (DCVAS). Clin Exp Immunol 164(Suppl 1):11–13. https://doi.org/10.1111/j.1365-2249.2011.04358.x

  30. 30.

    Watts RA (2019) Evolving concepts in classification of systemic vasculitis: where are we and what is the way forward? Int J Rheum Dis 22(Suppl 1):21–27. https://doi.org/10.1111/1756-185X.13304

Download references

Author information

LC-G had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. 1a. Substantial contributions to the conception or design of the work: LC-G; JP-M: JLL-M. 1b. Substantial contributions to acquisition of data for the work: EG-Á, IC-P; LP-G; IC; AG-F; AQ-M; ARL; DM-P; LC-G. 1c. Substantial contributions to analysis and interpretation of data for the work: LC-G; JP-M; JLL-M. 2. Drafting the article or revising it critically for important intelectual content: LC-G; EG-Á; IC-P; LP-G; IC; AG-F; AQ-M; ARL; DM-P; JLL-M; JP-M. 3. Final approval of the version of the article to be published: LC-G; EG-Á; IC-P; LP-G; IC; AG-F; AQ-M; ARL; DM-P; JLL-M; JP-M. 4. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: LC-G; EG-Á; IC-P; LP-G; IC; AG-F; AQ-M; ARL; DM-P; JLL-M; JP-M.

Correspondence to Luis Corral-Gudino.

Ethics declarations

Conflict of interest

Del-Pino-Montes, Javier: Consultancies, speaking fees, and honoraria (< 10,000$) Amgen, BMS, Celgene, FAES, Gebro, Janssen, Lilly, Novartis, Pfizer, Roche, Sanofi. The rest of authors declare that there is no conflict of interest.

Additional information

Publisher's Note

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

Congress abstract publication: There is no related congress abstract publication with the complete cohort. In 2015, an abstract was published with a previous version of the cohort (with data from 2002 to 2011 in three centers: Salamanca, Ourense, Ponferrada). Reference: “Could we do more with the same? Incorporating ANCA specificities to EMEA Algorithm could improve its usefulness on ANCA-associated vasculitis (AAV) recognition and prognosis”. 17th international ANCA vasculitis workshop. European Vasculitis Society. London. 20th April 2015.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 514 kb)

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

Corral-Gudino, L., González-Vázquez, E., Calero-Paniagua, I. et al. The complexity of classifying ANCA-associated small-vessel vasculitis in actual clinical practice: data from a multicenter retrospective survey. Rheumatol Int 40, 303–311 (2020). https://doi.org/10.1007/s00296-019-04406-5

Download citation

Keywords

  • Anti-neutrophil cytoplasmic antibody-associated vasculitis
  • Eosinophilic granulomatous with polyangiitis (Churg-Strauss)
  • Granulomatosis with polyangiitis
  • Microscopic polyangiitis
  • Classification
  • Prognosis