title: Analysis of a cluster of cases of Wegener granulomatosis.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Rheumatology Section, Department of Medicine, University of Pennsylvania, Rm. 503 Maloney Building, 3600 Spruce Street, Philadelphia, PA 19104, USA. albertd@mail.med.upenn.edu
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Albert
authors.forename: Alexis N
authors.initials: AN
authors.lastname: Albert
authors.forename: Melchiore
authors.initials: M
authors.lastname: Vernace
authors.forename: Jodi K
authors.initials: JK
authors.lastname: Sebastian
authors.forename: Elizabeth C
authors.initials: EC
authors.lastname: Hsia
mesh_terms: D002274:Carcinogens, Environmental / Q000009:adverse effects*; D002840:Chromates / Q000009:adverse effects; D016000:Cluster Analysis; D015331:Cohort Studies; D004000:Dichloroethylenes / Q000009:adverse effects; D004781:Environmental Exposure / Q000009:adverse effects*; D014890:Granulomatosis with Polyangiitis / Q000453:epidemiology / Q000209:etiology*; D006256:Hazardous Waste / Q000009:adverse effects*; D006801:Humans; D008738:Methyl Ethers / Q000009:adverse effects; D010414:Pennsylvania / Q000453:epidemiology; D011795:Surveys and Questionnaires; D014241:Trichloroethylene / Q000009:adverse effects; D014752:Vinyl Chloride / Q000009:adverse effects
chemical_list: D002274:Carcinogens, Environmental; D002840:Chromates; D004000:Dichloroethylenes; D006256:Hazardous Waste; D008738:Methyl Ethers; D014241:Trichloroethylene; C043243:methyl tert-butyl ether; C034944:chromic acid; D014752:Vinyl Chloride