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Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients
Imperial Coll London, England.
Imperial Coll London, England.
Gen University Hospital, Czech Republic; Charles University of Prague, Czech Republic.
Skånes University Hospital, Sweden.
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2017 (English)In: Kidney International, ISSN 0085-2538, E-ISSN 1523-1755, Vol. 92, no 3, 693-702 p.Article in journal (Refereed) Published
Abstract [en]

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.

Place, publisher, year, edition, pages
ELSEVIER SCIENCE INC , 2017. Vol. 92, no 3, 693-702 p.
Keyword [en]
anti-GBM disease; anti-neutrophil cytoplasm antibody; glomerulonephritis; Goodpasture syndrome; vasculitis
National Category
Urology and Nephrology
Identifiers
URN: urn:nbn:se:liu:diva-140038DOI: 10.1016/j.kint.2017.03.014ISI: 000407498200022PubMedID: 28506760OAI: oai:DiVA.org:liu-140038DiVA: diva2:1136717
Note

Funding Agencies|UK National Institute for Health Research (NIHR) Academic Clinical Lectureship; Wellcome Trust; NIHR Imperial Biomedical Research Centre; Charles University Hospital, Prague, Czech Republic [RVO-VFN64165]

Available from: 2017-08-29 Created: 2017-08-29 Last updated: 2017-09-14

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The full text will be freely available from 2018-05-12 15:04
Available from 2018-05-12 15:04

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Weiner, MariaSegelmark, Mårten
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Division of Drug ResearchFaculty of Medicine and Health SciencesDepartment of Nephrology
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