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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Bibtex

@article{7fe25b2460fd46ca9f89c5595eb804bc,
title = "Patients double-seropositive for ANCA and anti-GBM antibodies have varied renal survival, frequency of relapse, and outcomes compared to single-seropositive patients",
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.",
keywords = "Anti-GBM disease, Anti-neutrophil cytoplasm antibody, Glomerulonephritis, Goodpasture syndrome, Vasculitis",
author = "McAdoo, {Stephen P.} and Anisha Tanna and Zdenka Hruškov{\'a} and Lisa Holm and Maria Weiner and Nishkantha Arulkumaran and Amy Kang and Veronika Satrapov{\'a} and Jeremy Levy and Sophie Ohlsson and Vladimir Tesar and M{\aa}rten Segelmark and Pusey, {Charles D.}",
year = "2017",
month = "5",
day = "12",
doi = "10.1016/j.kint.2017.03.014",
language = "English",
journal = "Kidney International",
issn = "1523-1755",
publisher = "Nature Publishing Group",

}