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ANCA-associated vasculitis — clinical utility of using ANCA specificity to classify patients
- Source :
- Nature Reviews Rheumatology, Nature Reviews Rheumatology, Nature Publishing Group, 2016
- Publication Year :
- 2016
- Publisher :
- Springer Science and Business Media LLC, 2016.
-
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.
- Subjects :
- 0301 basic medicine
medicine.medical_specialty
Pathology
[SDV.IMM] Life Sciences [q-bio]/Immunology
Myeloblastin
Microscopic Polyangiitis
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Churg-Strauss Syndrome
urologic and male genital diseases
Sensitivity and Specificity
Antibodies, Antineutrophil Cytoplasmic
03 medical and health sciences
0302 clinical medicine
Rheumatology
Predictive Value of Tests
immune system diseases
Proteinase 3
Epidemiology
Eosinophilic
medicine
Humans
Immunologic Factors
cardiovascular diseases
skin and connective tissue diseases
ComputingMilieux_MISCELLANEOUS
Peroxidase
Inflammation
030203 arthritis & rheumatology
biology
business.industry
Granulomatosis with Polyangiitis
Prognosis
medicine.disease
respiratory tract diseases
3. Good health
030104 developmental biology
Myeloperoxidase
Predictive value of tests
Immunology
biology.protein
[SDV.IMM]Life Sciences [q-bio]/Immunology
Antibody
business
Granulomatosis with polyangiitis
Microscopic polyangiitis
Biomarkers
Subjects
Details
- ISSN :
- 17594804 and 17594790
- Volume :
- 12
- Database :
- OpenAIRE
- Journal :
- Nature Reviews Rheumatology
- Accession number :
- edsair.doi.dedup.....0be21588942878856661564d89e55983