ArticleKidney international2017
Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients.
Article in Kidney international, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 104 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
104 citing papers in PubMed, 1 synthesis or guideline pooled it, 227 citations in OpenAlex.
- Meta-Analytical Accuracy of ANCA Renal Risk Score for Prediction of Renal Outcome in Patients With ANCA-Associated Glomerulonephritis.Frontiers in medicine · 2021Pooled it
- Endopeptidase Cleavage of Anti-Glomerular Basement Membrane AntibodiesJournal of the American Society of Nephrology : JASN · 2022Trial
- Severe acute kidney failure due to double-seropositive anti-GBM glomerulonephritis in a child: a case for rituximab therapy.Pediatric nephrology (Berlin, Germany) · 2026Article
- Kidney Biopsy of a Patient With Double Positive Antiglomerular Basement Membrane and Myeloperoxidase-antineutrophil Cytoplasmic Antibodies.Kidney medicine · 2026Article
- Advances in the Diagnosis, Treatment and Prognosis of ANCA-Associated Glomerulonephritis.Medicina (Kaunas, Lithuania) · 2026Review
- Concurrent anti-GBM disease and p-ANCA vasculitis presenting as pulmonary-renal syndrome in a patient with chronic kidney disease: a case report.Annals of medicine and surgery (2012) · 2026Article
- Triple-Positive Serology in Rapidly Progressive Glomerulonephritis: A Diagnostic Dilemma.Kidney medicine · 2026Article
- Immunological overlap stratification in anti-GBM disease: prognostic differences and serological correlations-a single-center retrospective cohort study.Frontiers in immunology · 2026Article
- The effect of frequent plasmapheresis in a patient with anti-glomerular basement membrane antibody diseases with severe renal dysfunction: a case report and literature review.Frontiers in immunology · 2026Review
- Late post-transplant recurrence of an anti-myeloperoxydase antibody-associated vasculitis in a former double-positive patient: a case report.Frontiers in immunology · 2026Article
- Anti-GBM disease shortly after clinically presumed ANCA-associated vasculitis in an 85-year-old patient: a case report and literature review.Frontiers in nephrology · 2026Article
- Intersecting Autoimmunities: ANCA and Anti-GBM Overlap in a Patient With Sjögren's Disease.Case reports in nephrology · 2026Article
- Anti-glomerular basement membrane disease-treatment standard.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025Review
- Clinical profile and outcome of patients with ANCA-associated vasculitis with special reference to vasculitis damage index: a longitudinal observational study.Rheumatology international · 2025Observational
- Challenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis.Nature reviews. Rheumatology · 2025Review
- Clinical and pathological features of patients with recurrent anti-glomerular basement membrane disease.Renal failure · 2025Review
- Therapeutics controversies in antineutrophilic cytoplasmic antibody-associated vasculitis.Clinical and experimental nephrology · 2025Review
- Avacopan in Double-Positive Vasculitis...Why Not Consider It?: A Case Report.Kidney medicine · 2025Article
- Article
- Acute Kidney Failure in Limited Scleroderma Reveals Anti-GBM--Associated Kidney Disease.Kidney medicine · 2025Article
44 more citing papers are in PubMed but not listed here.
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Authors and funding
13 authors at 4 institutions in 3 countries.
Funding
Abstract
Co-presentation with both ANCA and anti-GBM antibodies is thought to be relatively rare. Current studies of such 'double-positive' cases report small numbers and variable outcomes. To study this further we retrospectively analyzed clinical features and long-term outcomes of a large cohort of 568 contemporary patients with ANCA-associated vasculitis, 41 patients with anti-GBM disease, and 37 double-positive patients with ANCA and anti-GBM disease from four European centers. Double-positive patients shared characteristics of ANCA-associated vasculitis (AAV), such as older age distribution and longer symptom duration before diagnosis, and features of anti-GBM disease, such as severe renal disease and high frequency of lung hemorrhage at presentation. Despite having more evidence of chronic injury on renal biopsy compared to patients with anti-GBM disease, double-positive patients had a greater tendency to recover from being dialysis-dependent after treatment and had intermediate long-term renal survival compared to the single-positive patients. However, overall patient survival was similar in all three groups. Predictors of poor patient survival included advanced age, severe renal failure, and lung hemorrhage at presentation. No single-positive anti-GBM patients experienced disease relapse, whereas approximately half of surviving patients with AAV and double-positive patients had recurrent disease during a median follow-up of 4.8 years. Thus, double-positive patients have a truly hybrid disease phenotype, requiring aggressive early treatment for anti-GBM disease, and careful long-term follow-up and consideration for maintenance immunosuppression for AAV. Since double-positivity appears common, further work is required to define the underlying mechanisms of this association and define optimum treatment strategies.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.