title: Outcomes of patients admitted to intensive care units for acute manifestation of small-vessel vasculitis: a multicenter, retrospective study.
abstract: BACKGROUND
  The outcomes of patients admitted to the intensive care unit (ICU) for acute manifestation of small-vessel vasculitis are poorly reported. The aim of the present study was to determine the mortality rate and prognostic factors of patients admitted to the ICU for acute small-vessel vasculitis.
  
  
  METHODS
  This retrospective, multicenter study was conducted from January 2001 to December 2014 in 20 ICUs in France. Patients were identified from computerized registers of each hospital using the International Classification of Diseases, Ninth Revision (ICD-9). Inclusion criteria were (1) known or highly suspected granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis (respectively, ICD-9 codes M31.3, M30.1, and M31.7), or anti-glomerular basement membrane antibody disease (ICD-9 codes N08.5X-005 or M31.0+); (2) admission to the ICU for the management of an acute manifestation of vasculitis; and (3) administration of a cyclophosphamide pulse in the ICU or within 48 h before admission to the ICU. The primary endpoint was assessment of mortality rate 90 days after admission to the ICU.
  
  
  RESULTS
  Eighty-two patients at 20 centers were included, 94% of whom had a recent (<6 months) diagnosis of small-vessel vasculitis. Forty-four patients (54%) had granulomatosis with polyangiitis. The main reasons for admission were respiratory failure (34%) and pulmonary-renal syndrome (33%). Mechanical ventilation was required in 51% of patients, catecholamines in 31%, and renal replacement therapy in 71%. Overall mortality at 90 days was 18% and the mortality in ICU was 16 %. The main causes of death in the ICU were disease flare in 69% and infection in 31%. In univariable analysis, relevant factors associated with death in nonsurvivors compared with survivors were Simplified Acute Physiology Score II (median [interquartile range] 51 [38-82] vs. 36 [27-42], p = 0.005), age (67 years [62-74] vs. 58 years [40-68], p < 0.003), Sequential Organ Failure Assessment score on the day of cyclophosphamide administration (11 [6-12] vs. 6 [3-7], p = 0.0004), and delayed administration of cyclophosphamide (5 days [3-14] vs. 2 days [1-5], p = 0.0053).
  
  
  CONCLUSIONS
  Patients admitted to the ICU for management of acute small-vessel vasculitis benefit from early, aggressive intensive care treatment, associated with an 18% death rate at 90 days.
authors.affiliation: Brabois Medical Intensive Care Unit, Nancy University Hospital, Vandoeuvre-les-Nancy, Nancy, 54000, France.|INSERM U1116, Vandoeuvre-les-Nancy, Nancy, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Kimmoun
authors.affiliation: Brabois Medical Intensive Care Unit, Nancy University Hospital, Vandoeuvre-les-Nancy, Nancy, 54000, France.|INSERM U1116, Vandoeuvre-les-Nancy, Nancy, France.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Baux
authors.affiliation: Medical-Surgical Intensive Care Unit, Andre Gregoire District Hospital Center, Montreuil, F-93105, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Das
authors.affiliation: Medical Intensive Care Unit, Caen University Hospital, Avenue de la Côte de Nacre, 14000, Caen, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Terzi
authors.affiliation: Medical Intensive Care Unit, Angers University Hospital, Angers, F-49933, France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Talec
authors.affiliation: Medical Intensive Care Unit, Angers University Hospital, Angers, F-49933, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Asfar
authors.affiliation: Medical Intensive Care Unit, Bretonneau University Hospital, Tours, F-37044, France.
authors.forename: Stephan
authors.initials: S
authors.lastname: Ehrmann
authors.affiliation: Medical Intensive Care Unit, Cochin University Hospital, Paris, F-75014, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Geri
authors.affiliation: Medical Intensive Care Unit, Rouen University Hospital, Rouen, 76031, France.
authors.forename: Steven
authors.initials: S
authors.lastname: Grange
authors.affiliation: Medical Intensive Care Unit, Kremlin-Bicêtre University Hospital, Paris, F-94275, France.
authors.forename: Nadia
authors.initials: N
authors.lastname: Anguel
authors.affiliation: Medical Intensive Care Unit and Respiratory Division, Pitié-Salpêtrière University Hospital, Paris, 75013, France.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Demoule
authors.affiliation: Medical-Surgical Intensive Care Unit, Lille University Hospital, Lille, F-59000, France.
authors.forename: Anne Sophie
authors.initials: AS
authors.lastname: Moreau
authors.affiliation: Medical Intensive Care Unit, Saint-Louis University Hospital, Paris, 75010, France.
authors.forename: Elie
authors.initials: E
authors.lastname: Azoulay
authors.affiliation: Medical Intensive Care Unit, Dijon University Hospital, Dijon, F-21079, France.
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Quenot
authors.affiliation: Medical Intensive Care Unit, NHC University Hospital, Strasbourg, F-67091, France.
authors.forename: Julie
authors.initials: J
authors.lastname: Boisramé-Helms
authors.affiliation: Medical Intensive Care Unit, Mercy Regional Hospital, Ars-Laquenexy, 57530, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Louis
authors.affiliation: Medical Intensive Care Unit, Bichat - Claude-Bernard University Hospital, Paris, 75018, France.
authors.forename: Romain
authors.initials: R
authors.lastname: Sonneville
authors.affiliation: INSERM CIC1433, Nancy University Hospital, Nancy, 54000, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Girerd
authors.affiliation: Brabois Medical Intensive Care Unit, Nancy University Hospital, Vandoeuvre-les-Nancy, Nancy, 54000, France.|INSERM U1116, Vandoeuvre-les-Nancy, Nancy, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Ducrocq
authors.affiliation: INSERM CIC-EC, CIE6, Nancy University Hospital, Nancy, 54000, France.
authors.forename: Nelly
authors.initials: N
authors.lastname: Agrinier
authors.affiliation: Vascular Medicine Division and Regional Competence Center for Rare Vascular and Systemic Autoimmune Diseases, Nancy University Hospital, Vandoeuvre-les Nancy, Nancy, 54511, France.
authors.forename: Denis
authors.initials: D
authors.lastname: Wahl
authors.affiliation: National Referral Center for Necrotizing Vasculitides and Systemic Sclerosis, Cochin Hospital, University Paris Descartes, Paris, F-75014, France.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: Brabois Medical Intensive Care Unit, Nancy University Hospital, Vandoeuvre-les-Nancy, Nancy, 54000, France. b.levy@chru-nancy.fr.|INSERM U1116, Vandoeuvre-les-Nancy, Nancy, France. b.levy@chru-nancy.fr.
authors.forename: Bruno
authors.initials: B
authors.lastname: Levy
mesh_terms: D000368:Aged; D005260:Female; D005602:France; D017052:Hospital Mortality; D006801:Humans; D007362:Intensive Care Units / Q000706:statistics & numerical data*; D008297:Male; D008875:Middle Aged; D063868:Patient Outcome Assessment; D011379:Prognosis; D012189:Retrospective Studies; D014657:Vasculitis / Q000401:mortality*
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