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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

McAdoo, SP; Tanna, A; Hruskova, Z; Holm, L; Weiner, M; Arulkumaran, N; Kang, A; ... Pusey, CD; + view all (2017) Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients. Kidney International , 92 (3) pp. 693-702. 10.1016/j.kint.2017.03.014. Green open access

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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.

Type: Article
Title: Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.kint.2017.03.014
Publisher version: http://dx.doi.org/10.1016/j.kint.2017.03.014
Language: English
Additional information: © 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/).
Keywords: 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
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Experimental and Translational Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10025456
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