title: Prevalence, correlates and outcomes of gastric antral vascular ectasia in systemic sclerosis: a EUSTAR case-control study.
abstract: OBJECTIVE
  To estimate the prevalence, determine the subgroups at risk, and the outcomes of patients with systemic sclerosis (SSc) and gastric antral vascular ectasia (GAVE).
  
  
  METHODS
  We queried the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) network for the recruitment of patients with SSc-GAVE. Each case was matched for cutaneous subset and disease duration with 2 controls with SSc recruited from the same center, evaluated at the time the index case made the diagnosis of GAVE. SSc characteristics were recorded at the time GAVE occurred and the last observation was collected to define the outcomes.
  
  
  RESULTS
  Forty-nine patients with SSc and GAVE were included (24 with diffuse cutaneous SSc) and compared to 93 controls with SSc. The prevalence of GAVE was estimated at about 1% of patients with SSc. By multivariate analysis, patients with SSc-GAVE more frequently exhibited a diminished (< 75%) DLCO value (OR 12.8; 95% CI 1.9-82.8) despite less frequent pulmonary fibrosis (OR 0.2; 95% CI 0.1-0.6). GAVE was also associated with the presence of anti-RNA-polymerase III antibodies (OR 4.6; 95% CI 1.2-21.1). SSc-GAVE was associated with anemia (82%) requiring blood transfusion (45%). Therapeutic endoscopic procedures were performed in 45% of patients with GAVE. After a median followup of 30 months (range 1-113 months), survival was similar in patients with SSc-GAVE compared to controls, but a higher number of scleroderma renal crisis cases occurred (12% vs 2%; p = 0.01).
  
  
  CONCLUSIONS
  GAVE is rare and associated with a vascular phenotype, including anti-RNA-polymerase III antibodies, and a high risk of renal crisis. Anemia, usually requiring blood transfusions, is a common complication.
authors.affiliation: From the Department of Rheumatology A, Paris Descartes University, Cochin Hospital, AP-HP, Paris, France; University of Michigan Scleroderma Program, Ann Arbor, Michigan, USA; Division of Rheumatology, University of Pennsylvania, Philadelphia, USA; Department of Rheumatology, University Hospital Zurich, Switzerland; Rheumatology and Clinical Immunology Unit, Spedali Civili and University of Brescia, Brescia, Italy; Rheumatology Department, Hospital Universitario 12 de Octubre, Madrid, Spain; Rheumatology Unit, Azienda Ospedaliera Universitaria V. Emanuele, Ferrarotto, S. Bambino, Catania, Italy; Service de Médecine Interne, Centre Hospitalier Nord, Marseille, France; Service de Médecine Interne, University Hospital Centre (CHU) Cochin; Service de Médecine Interne, CHU Saint-Antoine, Paris, France; Division of Rheumatology, Istituto Gaetano Pini, University of Milan, Italy; Department of Clinical Medicine, Clinical Immunology Unit, Sapienza University of Rome, Rome, Italy; Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata, Verona, Italia; Department of Rheumatology, Lund University, Lund, Sweden; Department of Rheumatology, Basel University, Basel, Switzerland; Sección de Reumatología, Hospital Dr Peset, Valencia, Spain; Médecine interne, Hôpital Beaujon, Clichy, France; Internal Medicine, Hôtel-Dieu Hospital, CHU Nantes, Nantes, France; Rheumatologic Unit, Department of Internal Medicine and Clinical Specialties, Sapienza University of Rome, Rome, Italy; and Department of Medicine, Georgetown University Medical Center, Washington DC, USA.
authors.forename: Etienne
authors.initials: E
authors.lastname: Ghrénassia
authors.forename: Jérome
authors.initials: J
authors.lastname: Avouac
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.forename: Chris T
authors.initials: CT
authors.lastname: Derk
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.forename: Yossra Atef
authors.initials: YA
authors.lastname: Suliman
authors.forename: Paolo
authors.initials: P
authors.lastname: Airo
authors.forename: Patricia E
authors.initials: PE
authors.lastname: Carreira
authors.forename: Rosario
authors.initials: R
authors.lastname: Foti
authors.forename: Brigitte
authors.initials: B
authors.lastname: Granel
authors.forename: Alice
authors.initials: A
authors.lastname: Berezne
authors.forename: Jean
authors.initials: J
authors.lastname: Cabane
authors.forename: Francesca
authors.initials: F
authors.lastname: Ingegnoli
authors.forename: Edoardo
authors.initials: E
authors.lastname: Rosato
authors.forename: Paola
authors.initials: P
authors.lastname: Caramaschi
authors.forename: Roger
authors.initials: R
authors.lastname: Hesselstrand
authors.forename: Ulrich A
authors.initials: UA
authors.lastname: Walker
authors.forename: Juan Jose
authors.initials: JJ
authors.lastname: Alegre-Sancho
authors.forename: Virginie
authors.initials: V
authors.lastname: Zarrouk
authors.forename: Christian
authors.initials: C
authors.lastname: Agard
authors.forename: Valeria
authors.initials: V
authors.lastname: Riccieri
authors.forename: Elena
authors.initials: E
authors.lastname: Schiopu
authors.forename: Heather
authors.initials: H
authors.lastname: Gladue
authors.forename: Virginia D
authors.initials: VD
authors.lastname: Steen
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
mesh_terms: D000328:Adult; D000368:Aged; D001803:Blood Transfusion; D016022:Case-Control Studies; D015897:Comorbidity; D016099:Endoscopy, Gastrointestinal; D005260:Female; D020252:Gastric Antral Vascular Ectasia / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery; D006488:Hemostasis, Surgical; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012306:Risk; D012595:Scleroderma, Systemic / Q000453:epidemiology*; D055815:Young Adult
keywords: GAVE; RNA POLYMERASE III; SCLERODERMA RENAL CRISIS; SYSTEMIC SCLEROSIS; WATERMELON STOMACH