Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients.
File(s)
Accepted version
Published version
Author(s)
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.
Date Issued
2017-05-12
Date Acceptance
2017-03-09
Citation
Kidney International, 2017, 92 (3), pp.693-702
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://
creativecommons.org/licenses/by/4.0/).
Elsevier Inc. This is an open access article under the CC BY license (http://
creativecommons.org/licenses/by/4.0/).
License URL
Sponsor
Imperial College Trust
Wellcome Trust
Imperial College Healthcare NHS Trust- BRC Funding
Vasculitis UK
The Academy of Medical Sciences
Identifier
PII: S0085-2538(17)30207-7
Grant Number
PC1997 01/09/01
097882/Z/11/ZR
RDA04 79560
ID 1503
N/A
Subjects
Science & Technology
Life Sciences & Biomedicine
Urology & Nephrology
anti-GBM disease
anti-neutrophil cytoplasm antibody
glomerulonephritis
Goodpasture syndrome
vasculitis
BASEMENT-MEMBRANE ANTIBODIES
ANTINEUTROPHIL CYTOPLASMIC ANTIBODY
RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS
CRESCENTIC GLOMERULONEPHRITIS
GOODPASTURES-DISEASE
MYELOPEROXIDASE
AUTOANTIBODIES
VASCULITIS
CLASSIFICATION
SPECIFICITY
anti–neutrophil cytoplasm antibody
1103 Clinical Sciences
Publication Status
Published