title: Granulomatosis with polyangiitis according to geographic origin and ethnicity: clinical-biological presentation and outcome in a French population.
abstract: Granulomatosis with polyangiitis (GPA) mainly affects white Europeans, but rarely GPA may also affect non-Europeans. This study aimed to describe GPA clinical-biological presentation and outcome in black sub-Saharan Africans and Afro-Caribbeans and in North Africans.
  
  
  
  Among 914 GPA patients included in the French Vasculitis Study Group database, geographic origin and ethnicity were known for 760. Clinical-biological presentations and outcomes of white Europeans vs black sub-Saharans and Afro-Caribbeans and vs North Africans were analysed.
  
  
  
  Among the 760 patients, 689 (91%) were white Europeans, 33 (4.3%) were North Africans and 22 (2.9%) were sub-Saharans (n = 8) or Afro-Caribbeans (French West Indies, n = 14). Black sub-Saharans and Afro-Caribbeans, compared with white Europeans, were significantly younger at GPA diagnosis (P = 0.003), had more frequent central nervous system involvement (P = 0.02), subglottic stenosis (P = 0.002) and pachymeningitis (P = 0.009), and tended to have more frequent chondritis and retroorbital tumour. Median serum creatinine levels and Birmingham Vasculitis Activity Score were significantly lower in sub-Saharans and Afro-Caribbeans (P = 0.002 and P = 0.003, respectively). In contrast, in comparison with white Europeans, North Africans had only less frequent arthralgias (P = 0.004). Time to relapse was shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans [adjusted HR = 1.96 (95% CI: 1.09, 3.51) (P = 0.02)], and did not differ for North Africans. In contrast, overall survival was not significantly different according to ethnicity.
  
  
  
  Our findings indicated different GPA clinical presentations in white Europeans and sub-Saharans and Afro-Caribbeans, with black patients having more frequent severe granulomatous manifestations. In addition, time to relapse was significantly shorter for black sub-Saharans and Afro-Caribbeans compared with white Europeans.
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (AP-HP).|Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.affiliation: Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité.|Centre de Recherche Epidémiologie et Statistique, INSERM U1153.|Centre d'Epidémiologie Clinique, Hôpital Hôtel-Dieu, AP-HP.
authors.forename: Agnès
authors.initials: A
authors.lastname: Dechartres
authors.affiliation: Department of Internal Medicine, CHU, Fort de France.
authors.forename: Christophe
authors.initials: C
authors.lastname: Deligny
authors.affiliation: Department of Internal Medicine, Centre Hospitalier Bretagne-Atlantique, Vannes.
authors.forename: Pascal
authors.initials: P
authors.lastname: Godmer
authors.affiliation: Department of Internal Medicine, Institut Mutualiste Montsouris, Paris.
authors.forename: Pierre
authors.initials: P
authors.lastname: Charles
authors.affiliation: Department of Rheumatology, Hôpital Ambroise Paré, AP-HP, Boulogne-Billancourt.
authors.forename: Gilles
authors.initials: G
authors.lastname: Hayem
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (AP-HP).|Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Dunogué
authors.affiliation: Department of Rheumatology, CHU, Fort de France, France.
authors.forename: Michel
authors.initials: M
authors.lastname: de Bandt
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (AP-HP).
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (AP-HP).
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (AP-HP).
authors.forename: Claire Le
authors.initials: CL
authors.lastname: Jeunne
authors.affiliation: Department of Internal Medicine, CHU, Fort de France.
authors.forename: Serge
authors.initials: S
authors.lastname: Arfi
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (AP-HP).|Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique - Hôpitaux de Paris (AP-HP).|Faculté de Médecine, Université Paris Descartes, Sorbonne Paris Cité.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000328:Adult; D017954:Africa South of the Sahara / Q000208:ethnology; D000352:Africa, Northern / Q000208:ethnology; D017677:Age Distribution; D000368:Aged; D044383:Black People / Q000208:ethnology; D002357:Cartilage Diseases / Q000208:ethnology* / Q000209:etiology; D003404:Creatinine / Q000097:blood; D005260:Female; D005602:France / Q000453:epidemiology; D014890:Granulomatosis with Polyangiitis / Q000097:blood / Q000150:complications / Q000208:ethnology* / Q000503:physiopathology; D006801:Humans; D007829:Laryngostenosis / Q000208:ethnology* / Q000209:etiology; D008297:Male; D008581:Meningitis / Q000208:ethnology* / Q000209:etiology; D008875:Middle Aged; D012008:Recurrence; D013997:Time Factors; D020293:Vasculitis, Central Nervous System / Q000208:ethnology* / Q000209:etiology; D014900:West Indies / Q000208:ethnology; D044465:White People / Q000208:ethnology
chemical_list: D003404:Creatinine
keywords: ANCA; black Afro-Caribbeans; black sub-Saharans; ethnicity; granulomatosis with polyangiitis; white Europeans