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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...
Autores principales: | , , , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
F1000 Research Limited
2022
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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 |
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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 |
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