Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a French urban multiethnic population in 2000: a capture-recapture estimate

Arthritis Rheum. 2004 Feb 15;51(1):92-9. doi: 10.1002/art.20077.


Objective: To estimate the prevalences of polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), Wegener's granulomatosis (WG), and Churg-Strauss syndrome (CSS).

Methods: Cases were collected in Seine-St. Denis County, a northeastern suburb of Paris, which has 1,093,515 adults (> or =15 years), 28% of whom are of non-European ancestry. The study period encompassed the entire calendar year 2000. Cases were identified by general practitioners, the departments of all the public hospitals and 2 large private clinics, and the National Health Insurance System. The Chapel Hill nomenclature was used to define MPA, and American College of Rheumatology criteria to define WG and CSS; PAN was diagnosed based on clinical laboratory, histological and/or angiographic findings. Three-source capture-recapture analysis was performed to correct for incomplete case ascertainment.

Results: A total of 75 cases were retained and capture-recapture analysis estimated that 23.8 cases had been missed by any 1 of the 3 sources. Accordingly, prevalences per 1,000,000 adults (95% confidence interval [95% CI]) were estimated to be 30.7 (95% CI 21-40) for PAN, 25.1 (95% CI 16-34) for MPA, 23.7 (95% CI 16-31) for WG, and 10.7 (95% CI 5-17) for CSS. The overall prevalence was 2.0 times higher for subjects of European ancestry than for non-Europeans (P = 0.01).

Conclusions: This study provides the first prevalence estimates for these 4 vasculitides for a multiethnic, urban population. The significantly higher prevalence observed for Europeans may infer a genetic susceptibility of Caucasians. Compared with previous estimates based mostly on rural populations, the higher frequency of PAN and the lower frequency of WG might suggest specific environmental etiologic factors.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age Distribution
  • Churg-Strauss Syndrome / diagnosis
  • Churg-Strauss Syndrome / epidemiology*
  • Female
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / epidemiology*
  • Humans
  • Male
  • Middle Aged
  • Paris / epidemiology
  • Polyarteritis Nodosa / diagnosis
  • Polyarteritis Nodosa / epidemiology*
  • Prevalence
  • Sex Ratio
  • Urban Health
  • White People / statistics & numerical data