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


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.

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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.

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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.

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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.

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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).

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