ArticleArthritis & rheumatology (Hoboken, N.J.)2016
Myeloperoxidase-Antineutrophil Cytoplasmic Antibody (ANCA)-Positive and ANCA-Negative Patients With Granulomatosis With Polyangiitis (Wegener's): Distinct Patient Subsets.
Article in Arthritis & rheumatology (Hoboken, N.J.), 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers, 1 of them a synthesis that pooled it.
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Who cites it
41 citing papers in PubMed, 1 synthesis or guideline pooled it, 105 citations in OpenAlex.
- Pooled it
- Primary and secondary vasculitis of the lung - critical evaluation of the update classification.Virchows Archiv : an international journal of pathology · 2026Article
- Challenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis.Nature reviews. Rheumatology · 2025Review
- A male patient with proptosis and palatal perforation: A diagnostic challenge.Radiology case reports · 2025Article
- Similarities and Differences Between Patients Diagnosed with ANCA-Associated Vasculitis Who Are Positive and Negative for ANCA: University Clinic Practice and Expertise.Medicina (Kaunas, Lithuania) · 2025Article
- Advances in the treatment of ANCA-associated vasculitis.Nature reviews. Rheumatology · 2025Review
- Age-Related Variations in the Clinical Presentation and Treatment Outcomes of New-Onset GPA: A Longitudinal Study.Journal of clinical medicine · 2025Article
- Relapse Predictors in Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis.Diagnostics (Basel, Switzerland) · 2024Review
- Dynamically changing antineutrophil cytoplasmic antibodies in granulomatosis with polyangiitis: A case report.World journal of clinical cases · 2024Article
- Article
- Clinical characteristics of hypertrophic cranial pachymeningitis in granulomatosis with polyangiitis: a retrospective single-center study in China.Arthritis research & therapy · 2024Article
- Granulomatosis with polyangiitis mimicking multisystem pyoderma gangrenosum: A case report.SAGE open medical case reports · 2024Article
- Article
- Significance of early treatment in granulomatosis with polyangiitis vasculitis.Clinical case reports · 2023Article
- Ocular Involvement of Granulomatosis with Polyangiitis.Journal of clinical medicine · 2023Article
- Article
- Article
- Interstitial lung disease in ANCA-associated vasculitis: associated factors, radiographic features and mortality.Rheumatology (Oxford, England) · 2023Article
- Case report: A pediatric case of MPO-ANCA-associated granulomatosis with polyangiitis superimposed on post-streptococcal acute glomerulonephritis.Frontiers in pediatrics · 2023Article
- Clinical profile and management of granulomatosis with polyangiitis-associated scleritis from a tertiary care hospital in South India.Indian journal of ophthalmology · 2023Article
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Authors and funding
16 authors at 10 institutions in 2 countries.
Funding
Abstract
objectiveTo examine the relationship of antineutrophil cytoplasmic antibody (ANCA) type and ANCA-associated vasculitis (AAV) diagnosis with demographic features, disease manifestations, and clinical outcomes. We focused on patients who account for the differences between ANCA type and disease type classifications: anti-myeloperoxidase (MPO)-ANCA-positive and ANCA-negative patients with granulomatosis with polyangiitis (Wegener's) (GPA).
methodsWe performed a pooled analysis of the Wegener's Granulomatosis Etanercept Trial and the Rituximab in ANCA-Associated Vasculitis trial comparing patients with MPO-ANCA-positive GPA and patients with ANCA-negative GPA to patients with proteinase 3 (PR3)-ANCA-positive GPA and patients with MPO-ANCA-positive microscopic polyangiitis (MPA).
resultsOf the 365 patients analyzed, 273 (75%) had PR3-ANCA-positive GPA, 33 (9%) had MPO-ANCA-positive GPA, 15 (4%) had ANCA-negative GPA, and 44 (12%) had MPO-ANCA-positive MPA. MPO-ANCA-positive GPA patients were younger at diagnosis compared to MPO-ANCA-positive MPA patients (53 versus 61 years; P = 0.02). Their disease manifestations and rates of relapse were similar to those of PR3-ANCA-positive GPA patients. Relapse was more frequent in MPO-ANCA-positive GPA patients than in patients with MPO-ANCA-positive MPA at trial entry as well as at 12 and 18 months. ANCA-negative patients with GPA had lower Birmingham Vasculitis Activity Score for Wegener's Granulomatosis scores at trial entry than PR3-ANCA-positive patients with GPA (4.5 versus 7.7; P < 0.01), primarily because of a lower prevalence of renal involvement.
conclusionWe were unable to demonstrate important clinical differences between MPO-ANCA-positive and PR3-ANCA-positive patients with GPA. The risk of relapse was associated more closely with disease type than with ANCA type in this patient cohort. These findings deserve consideration in the assessment of relapse risk in patients with AAV.
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