ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved]
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...
| Main Authors: | , , , , , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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F1000 Research Ltd
2022-12-01
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| Series: | HRB Open Research |
| Subjects: | |
| Online Access: | https://hrbopenresearch.org/articles/5-80/v1 |
| _version_ | 1827941115058192384 |
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| author | Shamma Al Nokhatha Eithne Nic an Ríogh Ted Fitzgerald Cliona Cowhig Louis Aslett Arthur White Matthew D. Griffin Michael R. Clarkson Alyssa Verrelli Declan DeFreitas Yvonne O’Meara John Holian Eamonn Molloy Liam Casserly Mark A. Little Sarah M. Moran Cathal Walsh Julie Power Jennifer Scott Niall Conlon |
| author_facet | Shamma Al Nokhatha Eithne Nic an Ríogh Ted Fitzgerald Cliona Cowhig Louis Aslett Arthur White Matthew D. Griffin Michael R. Clarkson Alyssa Verrelli Declan DeFreitas Yvonne O’Meara John Holian Eamonn Molloy Liam Casserly Mark A. Little Sarah M. Moran Cathal Walsh Julie Power Jennifer Scott Niall Conlon |
| author_sort | Shamma Al Nokhatha |
| collection | DOAJ |
| 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. |
| first_indexed | 2024-03-13T09:31:24Z |
| format | Article |
| id | doaj.art-5feb6a7cd1fb43caacda30430c4c53c3 |
| institution | Directory Open Access Journal |
| issn | 2515-4826 |
| language | English |
| last_indexed | 2024-03-13T09:31:24Z |
| publishDate | 2022-12-01 |
| publisher | F1000 Research Ltd |
| record_format | Article |
| series | HRB Open Research |
| spelling | doaj.art-5feb6a7cd1fb43caacda30430c4c53c32023-05-26T00:00:01ZengF1000 Research LtdHRB Open Research2515-48262022-12-01514929ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved]Shamma Al Nokhatha0Eithne Nic an Ríogh1https://orcid.org/0000-0002-8491-6504Ted Fitzgerald2Cliona Cowhig3Louis Aslett4Arthur White5Matthew D. Griffin6Michael R. Clarkson7Alyssa Verrelli8Declan DeFreitas9Yvonne O’Meara10John Holian11Eamonn Molloy12Liam Casserly13Mark A. Little14https://orcid.org/0000-0001-6003-397XSarah M. Moran15Cathal Walsh16https://orcid.org/0000-0001-6599-9562Julie Power17Jennifer Scott18https://orcid.org/0000-0001-8837-5250Niall Conlon19https://orcid.org/0000-0002-1102-0758Trinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, IrelandTrinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, IrelandTrinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, IrelandDepartment of Nephrology, Beaumont Hospital, Dublin, D09 V2N0, IrelandDepartment of Mathematical Sciences, Durham University, Durham, DH1 3LE, UKDepartment of Statistics, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, IrelandDepartment of Nephrology, University Hospital Galway, Galway, H91 YR71, IrelandDepartment of Nephrology, Cork University Hospital, Cork, T12 DC4A, IrelandDepartment of Nephrology, Cork University Hospital, Cork, T12 DC4A, IrelandDepartment of Nephrology, Beaumont Hospital, Dublin, D09 V2N0, IrelandDepartment of Nephrology, Mater Misericordiae University Hospital, Dublin, D07 R2WY, IrelandDepartment of Nephrology, St. Vincent’s University Hospital, Dublin, D04 T6F4, IrelandDepartment of Rheumatology, St. Vincent’s University Hospital, Dublin, D04 T6F4, IrelandDepartment of Nephrology, University Hospital Limerick, Limerick, V94 F858, IrelandTrinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, IrelandTrinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, IrelandDepartment of Mathematics and Statistics, University of Limerick, Limerick, V94 T9PX, IrelandVasculitis Ireland Awareness, Dublin, IrelandTrinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, IrelandDepartment of Immunology, St. James’s Hospital, Dublin, D08 NHY1, IrelandBackground: 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.https://hrbopenresearch.org/articles/5-80/v1ANCA-associated vasculitis registry outcomes death end-stage-kidney-disease urine soluble CD163 (usCD163)eng |
| spellingShingle | Shamma Al Nokhatha Eithne Nic an Ríogh Ted Fitzgerald Cliona Cowhig Louis Aslett Arthur White Matthew D. Griffin Michael R. Clarkson Alyssa Verrelli Declan DeFreitas Yvonne O’Meara John Holian Eamonn Molloy Liam Casserly Mark A. Little Sarah M. Moran Cathal Walsh Julie Power Jennifer Scott Niall Conlon ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved] HRB Open Research ANCA-associated vasculitis registry outcomes death end-stage-kidney-disease urine soluble CD163 (usCD163) eng |
| title | ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved] |
| title_full | ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved] |
| title_fullStr | ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved] |
| title_full_unstemmed | ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved] |
| title_short | ANCA-associated vasculitis in Ireland: a multi-centre national cohort study [version 1; peer review: 2 approved] |
| title_sort | anca associated vasculitis in ireland a multi centre national cohort study version 1 peer review 2 approved |
| topic | ANCA-associated vasculitis registry outcomes death end-stage-kidney-disease urine soluble CD163 (usCD163) eng |
| url | https://hrbopenresearch.org/articles/5-80/v1 |
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