Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients.

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Title: Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients.
Authors: McAdoo, SP
Tanna, A
Hrušková, Z
Holm, L
Weiner, M
Arulkumaran, N
Kang, A
Satrapová, V
Levy, J
Ohlsson, S
Tesar, V
Segelmark, M
Pusey, CD
Item Type: Journal Article
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.
Issue Date: 12-May-2017
Date of Acceptance: 9-Mar-2017
ISSN: 1523-1755
Publisher: Elsevier
Start Page: 693
End Page: 702
Journal / Book Title: Kidney International
Volume: 92
Issue: 3
Replaces: 10044/1/45556
Copyright Statement: © 2017 International Society of Nephrology. Published by Elsevier Inc. This is an open access article under the CC BY license (http://
Sponsor/Funder: Imperial College Trust
Wellcome Trust
Imperial College Healthcare NHS Trust- BRC Funding
Vasculitis UK
The Academy of Medical Sciences
Funder's Grant Number: PC1997 01/09/01
RDA04 79560
ID 1503
Keywords: Science & Technology
Life Sciences & Biomedicine
Urology & Nephrology
anti-GBM disease
anti-neutrophil cytoplasm antibody
Goodpasture syndrome
anti–neutrophil cytoplasm antibody
1103 Clinical Sciences
Publication Status: Published
Appears in Collections:Department of Medicine
Faculty of Medicine

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