Literature DB >> 16357755

Analysis of a cluster of cases of Wegener granulomatosis.

Daniel A Albert1, Alexis N Albert, Melchiore Vernace, Jodi K Sebastian, Elizabeth C Hsia.   

Abstract

BACKGROUND: Wegener granulomatosis is a chronic inflammatory autoimmune disease of unknown etiology. The sporadic occurrence, lack of familial or genetic associations, and rising incidence suggest possible exposure to environmental agents as causative for this disease.
OBJECTIVE: The objective of this study was to examine possible environmental triggers of Wegener granulomatosis.
METHODS: While conducting an environmental survey of potential precipitants of Wegener granulomatosis on a cohort of patients seen at Doylestown Hospital and at the University of Pennsylvania, we identified a cluster of cases in the Dublin, Pennsylvania, region. Through hospital records and patient contacts, we located 7 cases diagnosed in a 3-year period within a 10-mile radius of an Environmental Protection Agency (EPA) Superfund toxic waste site. The radius of inclusion represents a population of approximately 50,000 individuals. Assuming complete ascertainment of cases--which is unlikely given the methods used to acquire patients--the prevalence is 2- to 4-fold greater than the expected rate of 3 per 100,000. We identified toxins at or above "action level" within the demarcated geographic region using published data from the EPA. Furthermore, we queried patients regarding their particular chemical exposures.
RESULTS: These patients with Wegener granulomatosis were possibly exposed to high levels of trichloroethylene (TCE), vinyl chloride, methyl tertiary-butyl ether (MTBE), dichloroethene (DCE), and chromic acid from several industrial waste sites within the area. Additionally, these patients reported a total of greater than 30 possible exposures, including the aforesaid chemical contaminants. Three of 5 patients whose water source is known had well water that exposed them to industrial runoff and necessitated EPA intervention.
CONCLUSION: This data, along with other epidemiologic studies, suggest possible toxic exposures as potentially correctable risk factors for Wegener granulomatosis. We encourage clinicians to seek data that suggests an unusual environmental exposure and to solicit information that might implicate an industrial source for these exposures.

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Year:  2005        PMID: 16357755     DOI: 10.1097/01.rhu.0000173234.33984.4a

Source DB:  PubMed          Journal:  J Clin Rheumatol        ISSN: 1076-1608            Impact factor:   3.517


  5 in total

1.  Protein adducts of malondialdehyde and 4-hydroxynonenal contribute to trichloroethene-mediated autoimmunity via activating Th17 cells: dose- and time-response studies in female MRL+/+ mice.

Authors:  Gangduo Wang; Jianling Wang; Xiuzhen Fan; G A S Ansari; M Firoze Khan
Journal:  Toxicology       Date:  2011-12-09       Impact factor: 4.221

Review 2.  Organic solvents as risk factor for autoimmune diseases: a systematic review and meta-analysis.

Authors:  Carolina Barragán-Martínez; Cesar A Speck-Hernández; Gladis Montoya-Ortiz; Rubén D Mantilla; Juan-Manuel Anaya; Adriana Rojas-Villarraga
Journal:  PLoS One       Date:  2012-12-19       Impact factor: 3.240

3.  Bilateral idiopathic orbital inflammation 3 years before systemic Wegener's granulomatosis in a 7-year-old girl.

Authors:  Juan D Martínez-Gutiérrez; Enrique Mencía-Gutiérrez; Esperanza Gutiérrez-Díaz; José L Rodríguez-Peralto
Journal:  Clin Ophthalmol       Date:  2008-12

4.  Familial clusters of ANCA small-vessel vasculitis.

Authors:  Ana Marta Gomes; Filipe Nery; Ana Ventura; Clara Almeida; Joaquim Seabra
Journal:  NDT Plus       Date:  2008-10-31

Review 5.  Environmental factors influencing the risk of ANCA-associated vasculitis.

Authors:  Wen-Man Zhao; Zhi-Juan Wang; Rui Shi; Yu-Yu Zhu; Sen Zhang; Rui-Feng Wang; De-Guang Wang
Journal:  Front Immunol       Date:  2022-09-02       Impact factor: 8.786

  5 in total

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