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Mortality Rate by Diagnosis in ANCA-Associated Vasculitis Across FAIRVASC Registries

Tesi, Michelangelo ; Bettiol, Alessandra ; Gisslander, Karl LU orcid ; Mohammad, Aladdin J LU ; Hrušková, Zdenka ; Rutherford, Matthew ; Musial, Jacek ; Wojcik, krzysztof ; Little, Mark A. and Terrier, Benjamin , et al. (2024) p.187-187
Abstract
Background/ Objectives: Research into ANCA-associated vasculitis (AAV) is hampered by the rarity of the disease and the
subsequent small sample sizes of observational cohorts. This is further aggravated by the observational cohorts being contained
in fragmented data pools and lacking standardisation to allow for interoperability. FAIRVASC is a Europe-wide research project,
which has developed an infrastructure linking 7 existing AAV registries into a single European dataset, to allow high-quality
research on natural disease history and clinical outcomes.
This analysis highlights the capabilities of the FAIRVASC infrastructure to assess clinical outcome of AAV across the federated
registries.
Methods: Within the... (More)
Background/ Objectives: Research into ANCA-associated vasculitis (AAV) is hampered by the rarity of the disease and the
subsequent small sample sizes of observational cohorts. This is further aggravated by the observational cohorts being contained
in fragmented data pools and lacking standardisation to allow for interoperability. FAIRVASC is a Europe-wide research project,
which has developed an infrastructure linking 7 existing AAV registries into a single European dataset, to allow high-quality
research on natural disease history and clinical outcomes.
This analysis highlights the capabilities of the FAIRVASC infrastructure to assess clinical outcome of AAV across the federated
registries.
Methods: Within the FAIRVASC project, 7 national/regional AAV registries were harmonized using a semantic web approach,
including the creation of a dedicated AAV ontology, to enable semantic interoperability. For this study, aggregated data regarding
mortality rate (/100 person years, with related 95% Confidence Intervals) per diagnosis were retrieved through the FAIRVASC web
interface, a tool that allows federated querying over linked registries. We defined mortality rate in the first and second year post
diagnosis, and in years 3-5 and after 5 years.
Results: Mortality queries were posed over the FAIRVASC registries, namely RKD (Republic of Ireland, 677 patients), GFEV
(France, 2814 pts), ANCA (Czech Republic, 377 pts), PolVas (Poland, 944 pts), Skane (Sweden, 374 pts), Italivas (Italy, 301
pts), GeVas (Germany, 169 pts). Mortality rates, stratified by diagnosis and registry, are reported in Table 1. The lowest mortality
was reported for eosinophilic granulomatosis with polyangiitis (EGPA), with rates ranging from 0 to 2.5 cases/100 person-years
depending on the post-diagnosis interval. Conversely, the highest mortality was found for microscopic polyangiitis (MPA), with
mortality rates ranging from 6.7 to 22.3 across registries in the first year after diagnosis, and from 3.9 to 7.4 after >5 years from
diagnosis. In patients with granulomatosis with polyangiitis (GPA), mortality rates ranged from 3.3 to 8.2 in the first year after
diagnosis and from 0.7 to 4.5 after >5 years from diagnosis.
Conclusions: The FAIRVASC infrastructure is a reliable tool to query multiple AAV registries for assessing long-term clinical
outcomes of AAV in a large number of patients, in a privacy-compliant manner.
Disclosures: None. (Less)
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Contribution to conference
publication status
published
subject
pages
187 - 187
DOI
10.5281/zenodo.11068008
language
English
LU publication?
yes
id
54ee1ab9-5a52-4537-b43a-dae715a96cf7
date added to LUP
2024-09-13 17:09:43
date last changed
2024-09-16 09:05:08
@misc{54ee1ab9-5a52-4537-b43a-dae715a96cf7,
  abstract     = {{Background/ Objectives: Research into ANCA-associated vasculitis (AAV) is hampered by the rarity of the disease and the<br/>subsequent small sample sizes of observational cohorts. This is further aggravated by the observational cohorts being contained<br/>in fragmented data pools and lacking standardisation to allow for interoperability. FAIRVASC is a Europe-wide research project,<br/>which has developed an infrastructure linking 7 existing AAV registries into a single European dataset, to allow high-quality<br/>research on natural disease history and clinical outcomes.<br/>This analysis highlights the capabilities of the FAIRVASC infrastructure to assess clinical outcome of AAV across the federated<br/>registries.<br/>Methods: Within the FAIRVASC project, 7 national/regional AAV registries were harmonized using a semantic web approach,<br/>including the creation of a dedicated AAV ontology, to enable semantic interoperability. For this study, aggregated data regarding<br/>mortality rate (/100 person years, with related 95% Confidence Intervals) per diagnosis were retrieved through the FAIRVASC web<br/>interface, a tool that allows federated querying over linked registries. We defined mortality rate in the first and second year post<br/>diagnosis, and in years 3-5 and after 5 years.<br/>Results: Mortality queries were posed over the FAIRVASC registries, namely RKD (Republic of Ireland, 677 patients), GFEV<br/>(France, 2814 pts), ANCA (Czech Republic, 377 pts), PolVas (Poland, 944 pts), Skane (Sweden, 374 pts), Italivas (Italy, 301<br/>pts), GeVas (Germany, 169 pts). Mortality rates, stratified by diagnosis and registry, are reported in Table 1. The lowest mortality<br/>was reported for eosinophilic granulomatosis with polyangiitis (EGPA), with rates ranging from 0 to 2.5 cases/100 person-years<br/>depending on the post-diagnosis interval. Conversely, the highest mortality was found for microscopic polyangiitis (MPA), with<br/>mortality rates ranging from 6.7 to 22.3 across registries in the first year after diagnosis, and from 3.9 to 7.4 after &gt;5 years from<br/>diagnosis. In patients with granulomatosis with polyangiitis (GPA), mortality rates ranged from 3.3 to 8.2 in the first year after<br/>diagnosis and from 0.7 to 4.5 after &gt;5 years from diagnosis.<br/>Conclusions: The FAIRVASC infrastructure is a reliable tool to query multiple AAV registries for assessing long-term clinical<br/>outcomes of AAV in a large number of patients, in a privacy-compliant manner.<br/>Disclosures: None.}},
  author       = {{Tesi, Michelangelo and Bettiol, Alessandra and Gisslander, Karl and Mohammad, Aladdin J and Hrušková, Zdenka and Rutherford, Matthew and Musial, Jacek and Wojcik, krzysztof and Little, Mark A. and Terrier, Benjamin and Vaglio, Augusto and Emmi, Giacomo}},
  language     = {{eng}},
  month        = {{04}},
  pages        = {{187--187}},
  title        = {{Mortality Rate by Diagnosis in ANCA-Associated Vasculitis Across FAIRVASC Registries}},
  url          = {{http://dx.doi.org/10.5281/zenodo.11068008}},
  doi          = {{10.5281/zenodo.11068008}},
  year         = {{2024}},
}