ANCA-associated vasculitis-clinical utility of using ANCA specificity to classify patients

Divi Cornec, Emilie Cornec Le Gall, Fernando C. Fervenza, Ulrich Specks

Research output: Contribution to journalReview articlepeer-review

119 Scopus citations

Abstract

The anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are a heterogeneous group of rare syndromes characterized by necrotizing inflammation of small and medium-sized blood vessels and the presence of ANCAs. Several clinicopathological classification systems exist that aim to define homogeneous groups among patients with AAV, the main syndromes being microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). Two main types of ANCA can be detected in patients with AAV. These ANCAs are defined according to their autoantigen target, namely leukocyte proteinase 3 (PR3) and myeloperoxidase (MPO). Patients with GPA are predominantly PR3-ANCA-positive, whereas those with MPA are predominantly MPO-ANCA-positive, although ANCA specificity overlaps only partially with these clinical syndromes. Accumulating evidence suggests that ANCA specificity could be better than clinical diagnosis for defining homogeneous groups of patients, as PR3-ANCA and MPO-ANCA are associated with different genetic backgrounds and epidemiology. ANCA specificity affects the phenotype of clinical disease, as well as the patient's initial response to remission-inducing therapy, relapse risk and long-term prognosis. Thus, the classification of AAV by ANCA specificity rather than by clinical diagnosis could convey clinically useful information at the time of diagnosis.

Original languageEnglish (US)
Pages (from-to)570-579
Number of pages10
JournalNature Reviews Rheumatology
Volume12
Issue number10
DOIs
StatePublished - Oct 1 2016

ASJC Scopus subject areas

  • Rheumatology

Fingerprint

Dive into the research topics of 'ANCA-associated vasculitis-clinical utility of using ANCA specificity to classify patients'. Together they form a unique fingerprint.

Cite this