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

Abstract

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 (>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.

Association of HLA-DRB1*0901-DQB1*0303 haplotype with microscopic polyangiitis in Japanese

“…66.1711.4 years) and 77 unrelated healthy Japanese controls (46 men, 31 women, 25.975.6 years). Incidence of MPA in males and females appears to be approximately similar in Caucasians; 11,12 however, a nationwide study in Japan reported a female preponderance ANCA-associated vasculitis (male : female 1 : 1.8), among which MPA was most prevalent. 13 Diagnosis of MPA was based on the Japanese criteria.…”

“…11 Since HLA-DRB1*0901 is a common allele in Asian populations but rare in other populations, it was suggested that such a difference in the genetic background may be related to apparently higher prevalence of MPA in Japan as compared with Wegener's granulomatosis, which is more frequent in Caucasian populations. 2 Owing to the extensive linkage disequilibrium (LD) in the HLA region, statistical association with HLA-DRB1*0901 does not necessarily mean that HLA-DRB1*0901 molecule is involved in the pathogenesis of MPA.…”

Churg–Strauss syndrome: retrospective study in Burgundian population in France in past 10 years

“…The sex man/woman ratio is of 1.38. At diagnosis 50% of the patients live in the Côte d'Or department, 37.5% in the Saône-et-Loire, 8.5% in Nièvre and 4% in Yonne (p=0.02). In 48 % the patients live in rural zone in and in 52% in urban zone (p=NS).…”

“…Some minor forms have probably been followed only in a consultation or in outpatient clinic and are not recorded here. However, CSS is a rare pathology, multisystemic, seldom taken care of exclusively in outpatient [8]. These results are also superimposable with other important European series (incidence: 1-4/million inhabitants/ annum within the general population [9][10][11][12] and prevalence: 2-13/million inhabitants [12][13][14]), confirming the absence of North-South or East-West gradient within this pathology [13] or evolution in time [11] contrary to other vasculitis as the disease of Wegener whose incidence is currently in increase and who has a North-South decreasing gradient [15].…”

Polyarteriitis Nodosa with Real Arteries and Vein Involvement and Uroligical Manifestations: Case Report

“…The absence of hepatitis B, which is known to be strongly related with PAN, does not exclude the diagnosis. It was estimated that hepatitis B is causative for 30% and more recently -for 7-10% of PAN cases [6,7]. Given that, he met ACR criteria for PAN even before the kidney pathology became available.…”