The epidemiology of Wegener's granulomatosis. Estimates of the five‐year period prevalence, annual mortality, and geographic disease distribution from population‐based data sources

Cotch, Mary Frances; Hoffman, Gary S.; Yerg, Diane E.; Kaufman, Gerald I.; Targonski, Paul V.; Kaslow, Richard A.

doi:10.1002/art.1780390112

Cited by 294 publications

(169 citation statements)

References 9 publications

(1 reference statement)

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“…Nevertheless, a prevalence survey within a relatively genetically homogeneous population, the New Zealand European population, provides supportive evidence for a latitudinal gradient in WG that is not confounded by variation in ethnicity (8). Clearly, this current study provides no clues to explain the dearth of AAVs in individuals of African American descent (32,62); however, a similar pattern is seen in multiple sclerosis, another autoimmune disease with a striking latitudinal pattern of occurrence (63). One focus of future work could be to examine the role of ethnicity in the development of the AAVs.…”

Section: Association Between Uv Radiation and Wg Mpa And Cssmentioning

confidence: 68%

Antineutrophil cytoplasmic antibody–associated vasculitides: Could geographic patterns be explained by ambient ultraviolet radiation?

Gatenby

Lucas

Engelsen

et al. 2009

Arthritis & Rheumatism

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Objective. This ecological study describes and quantifies the association between ambient ultraviolet (UV) radiation levels, including daily winter vitamin D effective UV radiation levels and the incidence of the 3 antineutrophil cytoplasmic antibody-associated vasculitides (AAVs): Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS). Latitudinal variation in occurrence of the AAVs, especially WG, has been previously reported. For other autoimmune diseases such as multiple sclerosis and type 1 diabetes mellitus, inverse associations with latitude are hypothesized to indicate a causative role for low UV radiation exposure, possibly acting via vitamin D status. Methods. Published epidemiologic studies provided data on incident cases, total population of study regions, age-specific incidence rates, and study location. From these data and online age-specific population data, we calculated crude incidence rates, the expected number of cases (to control for possible age confounding), and measures of ambient UV radiation. Negative binomial regression models were used to calculate the incidence rate ratio (IRR) for a 1,000 joules/m 2 increase in ambient UV radiation.

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Section: Association Between Uv Radiation and Wg Mpa And Cssmentioning

confidence: 68%

Antineutrophil cytoplasmic antibody–associated vasculitides: Could geographic patterns be explained by ambient ultraviolet radiation?

Gatenby

Lucas

Engelsen

et al. 2009

Arthritis & Rheumatism

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“…Subsequent studies suggested that the number of men and women with this disease was approximately equal (2). At least 1 epidemiologic study has even reported a slightly higher percentage of women with the disease (29). In the first description of patients with limited WG (8), women outnumbered men at a ratio of 10 to 6.…”

Section: Discussionmentioning

confidence: 99%

Limited versus severe Wegener's granulomatosis: Baseline data on patients in the Wegener's granulomatosis etanercept trial

Stone¹

2003

Arthritis & Rheumatism

359

153

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Objective. To report baseline data on 180 patients with Wegener's granulomatosis (WG) enrolled in the WG Etanercept Trial (WGET), and to examine demographic and clinical differences between patients with limited disease and those with severe disease.Methods. Definitions of limited and severe disease were agreed upon by consensus of the investigators at a pretrial meeting and were incorporated into the protocol as a stratification criterion. These data were applied prospectively to the WGET patient cohort, based on clinical features and the intention to treat patients according to disease activity. Data related to disease onset, date of diagnosis, clinical features, antineutrophil cytoplasmic antibody assays, tissue biopsy findings, and other medical history were collected on a baseline medical history form. Physician-investigators from each center participated in the development of this form, and all were certified in its use prior to the start of the trial. Selected data on patients who were screened for the trial but were not enrolled were also collected.Results. Several significant differences between the limited and severe disease subsets were observed. Patients with limited disease were nearly a decade younger at disease onset compared with patients with severe disease. Thirty-three percent of patients with severe disease were women, compared with 58% of those with limited disease. Despite their younger age at symptom onset, patients with limited disease tended to have longer disease duration, a greater likelihood of experiencing exacerbation of previous disease following a period of remission, and a higher prevalence of destructive upper respiratory tract disorders at the time of enrollment (e.g., saddle-nose deformity). Patients with limited WG were less likely than those with severe disease to have antibodies to either proteinase 3 or myeloperoxidase. Patients with severe disease had a higher likelihood of previous thyroid disease, particularly either Graves' disease or Hashimoto thyroiditis, suggesting the possibility of different pathogenetic factors within these disease subsets. Other observed differences between these subsets, such as the greater frequency of alveolar hemorrhage in the severe disease group, were related to the a priori definitions of limited and severe disease.Conclusion. There are significant differences between patients with limited WG and those with severe WG with regard to sex, age, the likelihood of recurrent disease, the risk of damage in certain organ systems, and, possibly, etiologic factors. These differences (and perhaps other differences that are currently unrecognized) in patient subsets may have implications for mechanisms of pathogenesis, prognosis, response to treatment, and the design of future clinical investigations.

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“…The estimated prevalence of WG in Europe ranges from 2.4/100,000 to 15.7/100,000 (7). In the United States, the prevalence of WG has been estimated to be approximately 2.6/100,000 (8), and in Iran the prevalence of WG is about 0.06/100,000 in children under the age of 15 (9). However, the prevalence of WG in China has not been known until now.…”

Section: Introductionmentioning

confidence: 99%

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

Liu

Cui

et al. 2016

IRDR

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The epidemiology of Wegener's granulomatosis. Estimates of the five‐year period prevalence, annual mortality, and geographic disease distribution from population‐based data sources

Cited by 294 publications

References 9 publications

Antineutrophil cytoplasmic antibody–associated vasculitides: Could geographic patterns be explained by ambient ultraviolet radiation?

Antineutrophil cytoplasmic antibody–associated vasculitides: Could geographic patterns be explained by ambient ultraviolet radiation?

Limited versus severe Wegener's granulomatosis: Baseline data on patients in the Wegener's granulomatosis etanercept trial

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

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