title: Characteristics, prognosis, and outcomes of cutaneous ischemia and gangrene in systemic necrotizing vasculitides: a retrospective multicenter study.
abstract: OBJECTIVE
  To describe the prevalence, characteristics, and outcome of cutaneous ischemia, and whether it can occur in systemic necrotizing vasculitides (SNVs), i.e., polyarteritis nodosa, granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis.
  
  
  METHODS
  We conducted a retrospective analysis of all patients with SNV who were included in the French Vasculitis Study Group cohort database between March 1963 and September 2007. We compared characteristics and outcomes for patients with and without cutaneous ischemia (digital necrosis and/or isolated necrotic cutaneous ulcers).
  
  
  RESULTS
  Among the 1304 patients with a diagnosis of SNVs, 40 (3.1%) had digital necrosis and 25 (1.9%) had isolated necrotic cutaneous ulcers, with an equal distribution among SNVs. Presence of cutaneous ischemia was associated with past and/or current smoking [odds ratio (OR), 1.73; 95% confidence interval (95% CI), 1.02-2.95] and history of coronary artery disease (2.40; 1.01-6.00), as well as with other cutaneous manifestations (6.54; 3.21-8.67), gastrointestinal tract perforations (4.29; 1.41-13.07), and arthralgias (1.84; 1.10-3.07) during diagnosis. Ten patients with digital necrosis underwent extremity amputation, but no patient with isolated necrotic cutaneous ulcers (p = 0.007) underwent it. Smoking was the main risk factor of amputation (OR, 9.1; 1.7-48.9). At a mean follow-up of 10 years, cutaneous ischemia was identified as an independent predictor of vasculitis relapse (hazard ratio, 1.47; 95% CI, 1.05-2.05) and all-cause death (1.66; 1.01-2.74).
  
  
  CONCLUSIONS
  Cutaneous ischemia is a rare manifestation of SNVs but is associated with an increased risk of relapse and mortality. Tobacco use is associated with amputation, which emphasizes the importance of managing conventional cardiovascular risk factors in SNV patients.
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France; Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, 69495 Pierre-Bénite, Lyon, France. Electronic address: jean-christophe.lega@chu-lyon.fr.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Lega
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France.
authors.forename: Raphaèle
authors.initials: R
authors.lastname: Seror
authors.affiliation: Department of Internal and Vascular Medicine, Centre Hospitalier Lyon Sud, Hospices Civils de Lyon, Université Claude Bernard Lyon 1, 69495 Pierre-Bénite, Lyon, France.
authors.forename: Thomas
authors.initials: T
authors.lastname: Fassier
authors.affiliation: Department of Internal Medicine, Hôpital Gabriel-Montpied, CHU de Clermont-Ferrand, Clermont-Ferrand, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Aumaître
authors.affiliation: Department of Internal and Vascular Medicine, Hôpital Saint-Eloi, CHU de Montpellier France.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Quere
authors.affiliation: Department of Nephrology, Hôpital Rangueil, CHU de Toulouse, Toulouse, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Pourrat
authors.affiliation: Department of Internal Medicine, Centre Hospitalier de Verdun, Verdun, France.
authors.forename: Brigitte
authors.initials: B
authors.lastname: Gilson
authors.affiliation: Department of Dermatology, Hôpital Dupuytren, CHU de Limoges, Limoges, France.
authors.forename: Agnes
authors.initials: A
authors.lastname: Sparsa
authors.affiliation: Division of Internal and Vascular Medicine and Regional Competence Center for Rare Vascular And Systemic Autoimmune Diseases, CHU de Nancy; INSERM UMR_S 1116; Université de Lorraine, Nancy, France.
authors.forename: Denis
authors.initials: D
authors.lastname: Wahl
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Le Jeunne
authors.affiliation: Department of Internal Medicine, Hôpital Sud, CHU de Rennes, Rennes, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Decaux
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France.
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France.
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, Université Paris Descartes, Paris, France; Department of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005734:Gangrene / Q000453:epidemiology / Q000473:pathology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D012867:Skin / Q000098:blood supply* / Q000473:pathology; D056647:Systemic Vasculitis / Q000453:epidemiology / Q000473:pathology*
keywords: Cutaneous necrosis; Digital necrosis; Prognosis; Ulcers; Vasculitis