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Mortality in Wegener's granulomatosis: a bimodal pattern

Mortality in Wegener's granulomatosis: a bimodal pattern
Mortality in Wegener's granulomatosis: a bimodal pattern
Objective. To characterize the long-term mortality in patients with WG compared with matched population-based controls.

Methods. We used data from the General Practice Research Database, which contains the computerized records of 6.25 million patients and is representative of the population of the UK. We identified all subjects with a new diagnosis of WG in the period 1989–2004, and for each case, compared mortality with 10 controls matched for age, gender and practice.

Results. We identified 255 patients with a new diagnosis of WG (mean age 58.1 years, range 9–90 years, 47% females) and 2546 controls (mean age 58.1 years, range 9–89 years, 47% females). Mean follow-up was 6.4 years. The mortality for patients with WG was significantly increased during the first year after diagnosis [HR 9.0 (95% CI 5.8, 13.9)], especially for those ?65 years of age [HR 19.9 (95% CI 8.8, 44.9)]. The excess mortality was less marked after the first year: 1–5 years [HR 1.68 (95% CI 1.08, 2.60)], 5–10 years [HR 2.41 (95% CI 1.43, 4.07)], but started to increase by 10–15 years [HR 4.4 (95% CI 2.0, 9.8)]. The Kaplan–Meier survival curve showed an increase in mortality after 8 years.

Conclusions. Despite current therapy, patients with WG have a 9-fold increased risk of death in the first year of disease, attributed to infection, active vasculitis and renal failure. Between 1 and 8 years the risk is at its lowest, although higher than the control population. There is an increased mortality from 8 years onwards that remains unexplained.
wegener’s granulomatosis, mortality, vasculitis, general practice research database, cyclophosphamide, corticosteroids, prednisolone
1462-0324
697-702
Luqmani, Raashid
be06af41-8e76-4285-ab22-2b127992f153
Suppiah, Ravi
ea92a253-a8de-4647-bbe2-69cc065abfcb
Edwards, Christopher J.
dcb27fec-75ea-4575-a844-3588bcf14106
Phillip, Rhodri
1615c4af-9ab9-4c24-bcdc-eb1acbf2f657
Maskell, Joe
f10981fc-239d-4a91-acb6-d71cb9586529
Culliford, D.J.
25511573-74d3-422a-b0ee-dfe60f80df87
Jayne, David
ac68590a-ccc5-4b29-972a-39d502f4a5a1
Morishita, Kimberly
c2242fb6-032b-4404-9edb-06acfbc75d85
Arden, Nigel
23af958d-835c-4d79-be54-4bbe4c68077f
Luqmani, Raashid
be06af41-8e76-4285-ab22-2b127992f153
Suppiah, Ravi
ea92a253-a8de-4647-bbe2-69cc065abfcb
Edwards, Christopher J.
dcb27fec-75ea-4575-a844-3588bcf14106
Phillip, Rhodri
1615c4af-9ab9-4c24-bcdc-eb1acbf2f657
Maskell, Joe
f10981fc-239d-4a91-acb6-d71cb9586529
Culliford, D.J.
25511573-74d3-422a-b0ee-dfe60f80df87
Jayne, David
ac68590a-ccc5-4b29-972a-39d502f4a5a1
Morishita, Kimberly
c2242fb6-032b-4404-9edb-06acfbc75d85
Arden, Nigel
23af958d-835c-4d79-be54-4bbe4c68077f

Luqmani, Raashid, Suppiah, Ravi, Edwards, Christopher J., Phillip, Rhodri, Maskell, Joe, Culliford, D.J., Jayne, David, Morishita, Kimberly and Arden, Nigel (2011) Mortality in Wegener's granulomatosis: a bimodal pattern. Rheumatology, 50 (4), 697-702. (doi:10.1093/rheumatology/keq351). (PMID:21112869)

Record type: Article

Abstract

Objective. To characterize the long-term mortality in patients with WG compared with matched population-based controls.

Methods. We used data from the General Practice Research Database, which contains the computerized records of 6.25 million patients and is representative of the population of the UK. We identified all subjects with a new diagnosis of WG in the period 1989–2004, and for each case, compared mortality with 10 controls matched for age, gender and practice.

Results. We identified 255 patients with a new diagnosis of WG (mean age 58.1 years, range 9–90 years, 47% females) and 2546 controls (mean age 58.1 years, range 9–89 years, 47% females). Mean follow-up was 6.4 years. The mortality for patients with WG was significantly increased during the first year after diagnosis [HR 9.0 (95% CI 5.8, 13.9)], especially for those ?65 years of age [HR 19.9 (95% CI 8.8, 44.9)]. The excess mortality was less marked after the first year: 1–5 years [HR 1.68 (95% CI 1.08, 2.60)], 5–10 years [HR 2.41 (95% CI 1.43, 4.07)], but started to increase by 10–15 years [HR 4.4 (95% CI 2.0, 9.8)]. The Kaplan–Meier survival curve showed an increase in mortality after 8 years.

Conclusions. Despite current therapy, patients with WG have a 9-fold increased risk of death in the first year of disease, attributed to infection, active vasculitis and renal failure. Between 1 and 8 years the risk is at its lowest, although higher than the control population. There is an increased mortality from 8 years onwards that remains unexplained.

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More information

Published date: April 2011
Keywords: wegener’s granulomatosis, mortality, vasculitis, general practice research database, cyclophosphamide, corticosteroids, prednisolone
Organisations: Primary Care & Population Sciences

Identifiers

Local EPrints ID: 181799
URI: http://eprints.soton.ac.uk/id/eprint/181799
ISSN: 1462-0324
PURE UUID: d697f41a-9e1b-4f50-aefc-d902688d88a4
ORCID for D.J. Culliford: ORCID iD orcid.org/0000-0003-1663-0253

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Date deposited: 18 Apr 2011 10:31
Last modified: 15 Mar 2024 03:19

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Contributors

Author: Raashid Luqmani
Author: Ravi Suppiah
Author: Rhodri Phillip
Author: Joe Maskell
Author: D.J. Culliford ORCID iD
Author: David Jayne
Author: Kimberly Morishita
Author: Nigel Arden

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