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

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F17%3A10364263" target="_blank" >RIV/00216208:11110/17:10364263 - isvavai.cz</a>

Alternative codes found

RIV/00064165:_____/17:10364263

Result on the web

<a href="http://dx.doi.org/10.1016/j.kint.2017.03.014" target="_blank" >http://dx.doi.org/10.1016/j.kint.2017.03.014</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1016/j.kint.2017.03.014" target="_blank" >10.1016/j.kint.2017.03.014</a>

Result language

angličtina

Original language name

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

Original language description

Co-presentation with both ANCA and anti-GBM antibodies is thought to be relatively rare. Current studies of such &apos;double-positive&apos; 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.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

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OECD FORD branch

30217 - Urology and nephrology

Project

—

Continuities

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Publication year

2017

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Kidney International

ISSN

0085-2538

e-ISSN

—

Volume of the periodical

92

Issue of the periodical within the volume

3

Country of publishing house

US - UNITED STATES

Number of pages

10

Pages from-to

693-702

UT code for WoS article

000407498200022

EID of the result in the Scopus database

2-s2.0-85018861185