Literature DB >> 21112869

Mortality in Wegener's granulomatosis: a bimodal pattern.

Raashid Luqmani1, Ravi Suppiah, Christopher J Edwards, Rhodri Phillip, Joe Maskell, David Culliford, David Jayne, Kimberly Morishita, Nigel Arden.   

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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Year:  2010        PMID: 21112869     DOI: 10.1093/rheumatology/keq351

Source DB:  PubMed          Journal:  Rheumatology (Oxford)        ISSN: 1462-0324            Impact factor:   7.580


  27 in total

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Authors:  David R W Jayne; Annette N Bruchfeld; Lorraine Harper; Matthias Schaier; Michael C Venning; Patrick Hamilton; Volker Burst; Franziska Grundmann; Michel Jadoul; István Szombati; Vladimír Tesař; Mårten Segelmark; Antonia Potarca; Thomas J Schall; Pirow Bekker
Journal:  J Am Soc Nephrol       Date:  2017-04-11       Impact factor: 10.121

2.  Granulomatosis with polyangiitis (Wegener's granulomatosis): a rare variant of sudden natural death.

Authors:  Birthe Heitkötter; Cornelius Kuhnen; Sven Schmidt; Daniel Wittschieber
Journal:  Int J Legal Med       Date:  2017-10-31       Impact factor: 2.686

3.  Epidemiology and Outcomes of Granulomatosis With Polyangiitis in Pediatric and Working-Age Adult Populations In the United States: Analysis of a Large National Claims Database.

Authors:  Sirada Panupattanapong; Dustin L Stwalley; Andrew J White; Margaret A Olsen; Anthony R French; Mary E Hartman
Journal:  Arthritis Rheumatol       Date:  2018-12       Impact factor: 10.995

4.  The OMERACT core set of outcome measures for use in clinical trials of ANCA-associated vasculitis.

Authors:  Peter A Merkel; Sibel Z Aydin; Maarten Boers; Haner Direskeneli; Karen Herlyn; Philip Seo; Ravi Suppiah; Gunnar Tomasson; Raashid A Luqmani
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Review 5.  Maintenance of clinical remission in ANCA-associated vasculitis.

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6.  Using inpatient data to estimate the prevalence of Wegener's granulomatosis in China.

Authors:  Xiao Liu; Yazhou Cui; Yan Li; Chao Wang; Heng Zhao; Jinxiang Han
Journal:  Intractable Rare Dis Res       Date:  2016-02

7.  Changing patterns of clinical severity and risk of mortality in granulomatosis with polyangiitis over four decades: the Russian experience.

Authors:  Pavel I Novikov; Sergey V Moiseev; Ekaterina I Kuznetsova; Eugenia N Semenkova; Nikolay A Mukhin
Journal:  Rheumatol Int       Date:  2014-10-26       Impact factor: 2.631

8.  All-cause and cause-specific mortality in ANCA-associated vasculitis: overall and according to ANCA type.

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Journal:  Rheumatology (Oxford)       Date:  2020-09-01       Impact factor: 7.580

9.  Improved survival in granulomatosis with polyangiitis: A general population-based study.

Authors:  Zachary S Wallace; Na Lu; Sebastian Unizony; John H Stone; Hyon K Choi
Journal:  Semin Arthritis Rheum       Date:  2015-08-03       Impact factor: 5.532

Review 10.  Damage assessment in ANCA-associated vasculitis.

Authors:  Kuljeet Bhamra; Raashid Luqmani
Journal:  Curr Rheumatol Rep       Date:  2012-12       Impact factor: 4.592

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