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

Autor:	Scott J; Trinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, Ireland., Nic An Ríogh E; Trinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, Ireland., Al Nokhatha S; Trinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, Ireland., Cowhig C; Department of Nephrology, Beaumont Hospital, Dublin, D09 V2N0, Ireland., Verrelli A; Department of Nephrology, Cork University Hospital, Cork, T12 DC4A, Ireland., Fitzgerald T; Trinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, Ireland.; Department of Nephrology, Beaumont Hospital, Dublin, D09 V2N0, Ireland., White A; Department of Statistics, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, Ireland., Walsh C; Department of Mathematics and Statistics, University of Limerick, Limerick, V94 T9PX, Ireland., Aslett L; Department of Mathematical Sciences, Durham University, Durham, DH1 3LE, UK., DeFreitas D; Department of Nephrology, Beaumont Hospital, Dublin, D09 V2N0, Ireland., Clarkson MR; Department of Nephrology, Cork University Hospital, Cork, T12 DC4A, Ireland., Holian J; Department of Nephrology, St. Vincent's University Hospital, Dublin, D04 T6F4, Ireland., Griffin MD; Department of Nephrology, University Hospital Galway, Galway, H91 YR71, Ireland., Conlon N; Department of Immunology, St. James's Hospital, Dublin, D08 NHY1, Ireland., O'Meara Y; Department of Nephrology, Mater Misericordiae University Hospital, Dublin, D07 R2WY, Ireland., Casserly L; Department of Nephrology, University Hospital Limerick, Limerick, V94 F858, Ireland., Molloy E; Department of Rheumatology, St. Vincent's University Hospital, Dublin, D04 T6F4, Ireland., Power J; Vasculitis Ireland Awareness, Dublin, Ireland., Moran SM; Trinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, Ireland.; Department of Nephrology, Cork University Hospital, Cork, T12 DC4A, Ireland., Little MA; Trinity Health Kidney Centre, Trinity College Dublin, The University of Dublin, Dublin, D02 PN40, Ireland.
Jazyk:	angličtina
Zdroj:	HRB open research [HRB Open Res] 2022 Dec 01; Vol. 5, pp. 80. Date of Electronic Publication: 2022 Dec 01 (Print Publication: 2022).
DOI:	10.12688/hrbopenres.13651.1
Abstrakt:	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. Competing Interests: No competing interests were disclosed. (Copyright: © 2022 Scott J et al.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu