Cargando…

ANCA-associated vasculitis in Ireland: a multi-centre national cohort study

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare multisystem autoimmune disease. There is a need for interoperable national registries to enable reporting of real-world long-term outcomes and their predictors in AAV. Methods: The Irish National Rare Kidney...

Descripción completa

Detalles Bibliográficos
Autores principales: Scott, Jennifer, Nic an Ríogh, Eithne, Al Nokhatha, Shamma, Cowhig, Cliona, Verrelli, Alyssa, Fitzgerald, Ted, White, Arthur, Walsh, Cathal, Aslett, Louis, DeFreitas, Declan, Clarkson, Michael R., Holian, John, Griffin, Matthew D., Conlon, Niall, O’Meara, Yvonne, Casserly, Liam, Molloy, Eamonn, Power, Julie, Moran, Sarah M., Little, Mark A.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: F1000 Research Limited 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10213823/
https://www.ncbi.nlm.nih.gov/pubmed/37251362
http://dx.doi.org/10.12688/hrbopenres.13651.1
_version_ 1785047711022579712
author Scott, Jennifer
Nic an Ríogh, Eithne
Al Nokhatha, Shamma
Cowhig, Cliona
Verrelli, Alyssa
Fitzgerald, Ted
White, Arthur
Walsh, Cathal
Aslett, Louis
DeFreitas, Declan
Clarkson, Michael R.
Holian, John
Griffin, Matthew D.
Conlon, Niall
O’Meara, Yvonne
Casserly, Liam
Molloy, Eamonn
Power, Julie
Moran, Sarah M.
Little, Mark A.
author_facet Scott, Jennifer
Nic an Ríogh, Eithne
Al Nokhatha, Shamma
Cowhig, Cliona
Verrelli, Alyssa
Fitzgerald, Ted
White, Arthur
Walsh, Cathal
Aslett, Louis
DeFreitas, Declan
Clarkson, Michael R.
Holian, John
Griffin, Matthew D.
Conlon, Niall
O’Meara, Yvonne
Casserly, Liam
Molloy, Eamonn
Power, Julie
Moran, Sarah M.
Little, Mark A.
author_sort Scott, Jennifer
collection PubMed
description Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare multisystem autoimmune disease. There is a need for interoperable national registries to enable reporting of real-world long-term outcomes and their predictors in AAV. Methods: The Irish National Rare Kidney Disease (RKD) registry was founded in 2012. To date, 842 patients with various forms of vasculitis have been recruited across eight nephrology, rheumatology and immunology centres. We focus here on patient- and disease- characteristics, treatment and outcomes of the 397 prospectively recruited patients with AAV. Results: Median age was 64 years (IQR 55–73), 57.9% were male, 58.9% had microscopic polyangiitis and 85.9% had renal impairment. Cumulative one- and five-year patient survival was 94% and 77% respectively. Median follow-up was 33.5 months (IQR 10.7–52.7). After controlling for age, baseline renal dysfunction (p = 0.04) and the burden of adverse events (p <0.001) were independent predictors of death overall. End-stage-kidney-disease (ESKD) occurred in 73 (18.4%) patients; one- and five-year renal survival was 85% and 79% respectively. Baseline severity of renal insufficiency (p = 0.02), urine soluble CD163 (usCD163) (p = 0.002) and “sclerotic” Berden histological class (p = 0.001) were key determinants of ESKD risk. Conclusions: Long-term outcomes of Irish AAV patients are comparable to other reported series. Our results emphasise the need for personalisation of immunosuppression, to limit treatment toxicity, particularly in those with advanced age and renal insufficiency. Baseline usCD163 is a potential biomarker for ESKD prediction and should be validated in a large independent cohort.
format Online
Article
Text
id pubmed-10213823
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher F1000 Research Limited
record_format MEDLINE/PubMed
spelling pubmed-102138232023-05-27 ANCA-associated vasculitis in Ireland: a multi-centre national cohort study Scott, Jennifer Nic an Ríogh, Eithne Al Nokhatha, Shamma Cowhig, Cliona Verrelli, Alyssa Fitzgerald, Ted White, Arthur Walsh, Cathal Aslett, Louis DeFreitas, Declan Clarkson, Michael R. Holian, John Griffin, Matthew D. Conlon, Niall O’Meara, Yvonne Casserly, Liam Molloy, Eamonn Power, Julie Moran, Sarah M. Little, Mark A. HRB Open Res Research Article Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare multisystem autoimmune disease. There is a need for interoperable national registries to enable reporting of real-world long-term outcomes and their predictors in AAV. Methods: The Irish National Rare Kidney Disease (RKD) registry was founded in 2012. To date, 842 patients with various forms of vasculitis have been recruited across eight nephrology, rheumatology and immunology centres. We focus here on patient- and disease- characteristics, treatment and outcomes of the 397 prospectively recruited patients with AAV. Results: Median age was 64 years (IQR 55–73), 57.9% were male, 58.9% had microscopic polyangiitis and 85.9% had renal impairment. Cumulative one- and five-year patient survival was 94% and 77% respectively. Median follow-up was 33.5 months (IQR 10.7–52.7). After controlling for age, baseline renal dysfunction (p = 0.04) and the burden of adverse events (p <0.001) were independent predictors of death overall. End-stage-kidney-disease (ESKD) occurred in 73 (18.4%) patients; one- and five-year renal survival was 85% and 79% respectively. Baseline severity of renal insufficiency (p = 0.02), urine soluble CD163 (usCD163) (p = 0.002) and “sclerotic” Berden histological class (p = 0.001) were key determinants of ESKD risk. Conclusions: Long-term outcomes of Irish AAV patients are comparable to other reported series. Our results emphasise the need for personalisation of immunosuppression, to limit treatment toxicity, particularly in those with advanced age and renal insufficiency. Baseline usCD163 is a potential biomarker for ESKD prediction and should be validated in a large independent cohort. F1000 Research Limited 2022-12-01 /pmc/articles/PMC10213823/ /pubmed/37251362 http://dx.doi.org/10.12688/hrbopenres.13651.1 Text en Copyright: © 2022 Scott J et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution Licence, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
Scott, Jennifer
Nic an Ríogh, Eithne
Al Nokhatha, Shamma
Cowhig, Cliona
Verrelli, Alyssa
Fitzgerald, Ted
White, Arthur
Walsh, Cathal
Aslett, Louis
DeFreitas, Declan
Clarkson, Michael R.
Holian, John
Griffin, Matthew D.
Conlon, Niall
O’Meara, Yvonne
Casserly, Liam
Molloy, Eamonn
Power, Julie
Moran, Sarah M.
Little, Mark A.
ANCA-associated vasculitis in Ireland: a multi-centre national cohort study
title ANCA-associated vasculitis in Ireland: a multi-centre national cohort study
title_full ANCA-associated vasculitis in Ireland: a multi-centre national cohort study
title_fullStr ANCA-associated vasculitis in Ireland: a multi-centre national cohort study
title_full_unstemmed ANCA-associated vasculitis in Ireland: a multi-centre national cohort study
title_short ANCA-associated vasculitis in Ireland: a multi-centre national cohort study
title_sort anca-associated vasculitis in ireland: a multi-centre national cohort study
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10213823/
https://www.ncbi.nlm.nih.gov/pubmed/37251362
http://dx.doi.org/10.12688/hrbopenres.13651.1
work_keys_str_mv AT scottjennifer ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT nicanriogheithne ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT alnokhathashamma ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT cowhigcliona ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT verrellialyssa ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT fitzgeraldted ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT whitearthur ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT walshcathal ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT aslettlouis ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT defreitasdeclan ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT clarksonmichaelr ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT holianjohn ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT griffinmatthewd ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT conlonniall ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT omearayvonne ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT casserlyliam ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT molloyeamonn ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT powerjulie ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT moransarahm ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy
AT littlemarka ancaassociatedvasculitisinirelandamulticentrenationalcohortstudy