ArticleClinical kidney journal2021
Slowly progressive anti-neutrophil cytoplasmic antibody-associated renal vasculitis: clinico-pathological characterization and outcome.
Article in Clinical kidney journal, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 32 citations in OpenAlex.
- Advances in the Diagnosis, Treatment and Prognosis of ANCA-Associated Glomerulonephritis.Medicina (Kaunas, Lithuania) · 2026Review
- Kidney Outcomes in ANCA-Glomerulonephritis According to Induction Immunosuppression and Histopathology.Kidney international reports · 2026Article
- Development of Nephritis and Abnormal Lung Shadow in a Patient with IgG4-related Hypertrophic Pachymeningitis with MPO-ANCA Positivity after Unplanned Interruption of Maintenance Glucocorticoid Therapy.Internal medicine (Tokyo, Japan) · 2026Article
- Dual MPO-ANCA and PLA2R positivity in crescentic glomerulonephritis with MPO-associated membranous nephropathy: a case report with pathogenic insights.CEN case reports · 2026Article
- Advances in the treatment of ANCA-associated vasculitis.Nature reviews. Rheumatology · 2025Review
- Prevalence and characteristics of bronchiectasis in ANCA-associated vasculitis: A systematic review and meta-analysis.Archives of rheumatology · 2024Review
- Presentation and progression of MPO-ANCA interstitial lung disease.Journal of translational autoimmunity · 2024Article
- Very Slowly Progressive Microscopic Polyangiitis: Comparative Analysis with Rapidly Progressive Forms.Cureus · 2024Article
- Slowly Progressive ANCA-associated Glomerulonephritis with Strong Mesangial MPO Deposits Following a Diagnosis of Interstitial Lung Disease.Internal medicine (Tokyo, Japan) · 2024Article
- Changing Phenotypes and Clinical Outcomes Over Time in Microscopic Polyangiitis.Kidney international reports · 2023Article
- Outcomes in ANCA-Associated Vasculitis in Scotland: Validation of the Renal Risk Score in a Complete National Cohort.Kidney international reports · 2023Article
- Challenges of defining renal response in ANCA-associated vasculitis: call to action?Clinical kidney journal · 2023Review
- Article
- Genetics of ANCA-associated vasculitis: role in pathogenesis, classification and management.Nature reviews. Rheumatology · 2022Review
- A case of smoldering antineutrophil cytoplasmic antibody-associated vasculitis development during the course of primary Sjögren's syndrome.CEN case reports · 2022Article
- ANCA Status or Clinical Phenotype - What Counts More?Current rheumatology reports · 2021Review
- Slo-Mo anti-neutrophil cytoplasmic antibody-associated renal vasculitis.Clinical kidney journal · 2021Article
- Shifting Sands or ANCA-ed in Place? The Epidemiology of ANCA-Associated Vasculitis over 2 Decades at a Large Tertiary Centre.Glomerular diseasesArticle
- Interest of a Kidney Biopsy to Rule out ANCA-Associated Renal Vasculitis in Glomerulonephritis Patients with a Positive ANCA.Case reports in nephrology and dialysisArticle
Corrections and comments
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Authors and funding
21 authors at 11 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAlthough rapidly progressive glomerulonephritis is the main renal phenotype of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), slow renal disease progression is sometimes observed. These forms have been rarely discussed; we analysed their prevalence, clinico-pathological characteristics and outcome.
methodsWe screened patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis followed at seven referral centres and selected those with estimated glomerular filtration rate (eGFR) reduction <50% over a 6-month period preceding diagnosis. Data regarding patient features and response to treatment were retrieved.
resultsOf 856 patients, 41 (5%) had slowly progressive renal AAV. All had MPA and all but one was P-ANCA/myeloperoxidase (MPO) ANCA-positive. At diagnosis, the median age was 70 years [interquartile range (IQR) 64-78] and extra-renal manifestations were absent or subclinical (interstitial lung lesions in 10, 24%). The median (IQR) eGFR was 23 mL/min/1.73 m
conclusionsAAV may present with slow renal disease progression; this subset is hallmarked by advanced age at diagnosis, positive MPO-ANCA, subclinical interstitial lung lesions and chronic damage at kidney biopsy. Partial renal recovery may occur following immunosuppression.
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