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Increasing Incidence of Wegener´s granulomatosis in Sweden 1975-2001
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences. (Rheumatology)
2006 (English)In: Journal of Rheumatology, ISSN 0315-162X, E-ISSN 1499-2752, Vol. 33, no 10, 2060-2063 p.Article in journal (Refereed) Published
Abstract [en]

Objective. Studies of Wegener's granulomatosis (WG) since the late 1980s indicate a probable increase in incidence of unknown cause and significance, possibly related to antineutrophil cytoplasmic antibody (ANCA) testing. To extend these observations and to include calendar periods before ANCA was introduced, we assessed time trends in the incidence of WG in Sweden in the period 1975-2001.

Methods. In the population-based Swedish Inpatient Register, we identified 1938 individuals diagnosed with WG in the period 1975-2001, and calculated the annual age and sex adjusted incidences.

Results. The incidence increased from 0.33 (95% CI 0.28-0.39) in the period 1975-85 to 0.77 (95% CI 0.69-0.85) in 1986-90, to 1.19 (95% CI 1.12-1.26) in 1991-2001, resulting in a mean incidence of 0.78 (95% CI 0.74-0.82).

Conclusion. WG displays a strong temporal trend. While the increase coincides to some extent with the implementation of ANCA testing, suggestive of increased disease recognition, ANCA testing remains an incomplete explanation as increasing incidences were noted before as well as after their introduction.

Place, publisher, year, edition, pages
2006. Vol. 33, no 10, 2060-2063 p.
Keyword [en]
Wegener's granulomatosis, incidence, Sweden, antineutrophil cytoplasmic antibodies
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-95892ISI: 000241275200026PubMedID: 16960922OAI: oai:DiVA.org:uu-95892DiVA: diva2:170263
Available from: 2007-05-10 Created: 2007-05-10 Last updated: 2011-05-20Bibliographically approved
In thesis
1. Clinical and Epidemiological Studies of Wegener´s Granulomatosis
Open this publication in new window or tab >>Clinical and Epidemiological Studies of Wegener´s Granulomatosis
2007 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Wegener´s granulomatosis (WG) is an unusual, serious, systemic vasculitis with specific clinical findings. The studies in this thesis aim at broadening our understanding of the aetiology and outcome of WG.

Patients with WG were identified in the In-patient Register 1975-2001. During this time the incidence increased three-fold, and neither ANCA-related increased awareness, nor diagnostic drift, seem to fully explain this trend, but it is still unclear if a true rise in incidence exists.

Anti- neutrophil cytoplasmic antibodies (ANCA) have been presented as highly specific for vasculitis. In a series of consecutive cANCA/PR3-ANCA positive patients, we investigated the positive predictive value for ANCA, and the outcome of patients with a positive cANCA/PR3-ANCA but not vasculitis. These patients have a low future risk of developing vasculitis, possibly indicating that ANCA, in this setting, reflects neutrophil activating properties not specific to vasculitis.

By linkage of the WG-cohort, and randomly selected population controls, to the Multi-generation register, we identified all first-degree relatives and spouses of patients and controls, totally encompassing some 2,000 patients and 70,000 relatives. Familial aggregation of WG was the exception, with absolute risks of < 1 per 1000.However, relative risks in first-grade relatives amounted to 1.56 (95% CI 0.35-6.90) such that a moderate familial aggregation cannot be excluded.

In the WG-cohort, cancer occurrence and risk was compared to that of the general population. Patients with WG have an overall doubled risk of cancer, with particularly increased risks of bladder-cancer, haematopoietic cancers including lymphomas and squamous skin-cancer. In a case-control study nested within the WG-cohort, treatment with cyclophosphamide was compared among bladder-cancer patients and matched cancer-free controls. Absolute risk of bladder cancer as high as 10% some years after diagnosis were found, and this risk can partly be attributed to cyclophosphamide-treatment, with a dose-response relationship.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2007. 80 p.
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 258
Medicine, Wegener´s granulomatosis, incidence, time-trends, ANCA, familial aggregation, cancer risks, bladder cancer, cyclophosphamide, Medicin
urn:nbn:se:uu:diva-7887 (URN)978-91-554-6892-7 (ISBN)
Public defence
2007-05-31, Robergsalen, Ingång 40, Akademiska Sjukhuset, Uppsala, 09:15
Available from: 2007-05-10 Created: 2007-05-10 Last updated: 2013-09-19Bibliographically approved

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