Prevalence, correlates and outcomes of gastric antral vascular ectasia in systemic sclerosis: A eustar case-control study

Etienne Ghrénassia, Jérome Avouac, Dinesh Khanna, Chris T. Derk, Oliver Distler, Yossra Atef Suliman, Paolo Airo, Patricia E. Carreira, Rosario Foti, Brigitte Granel, Alice Berezne, Jean Cabane, Francesca Ingegnoli, Edoardo Rosato, Paola Caramaschi, Roger Hesselstrand, Ulrich A. Walker, Juan Jose Alegre-Sancho, Virginie Zarrouk, Christian AgardValeria Riccieri, Elena Schiopu, Heather Gladue, Virginia D. Steen, Yannick Allanore

Research output: Contribution to journalArticlepeer-review

64 Scopus citations


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). Conclusion. 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. The Journal of Rheumatology

Original languageEnglish (US)
Pages (from-to)99-105
Number of pages7
JournalJournal of Rheumatology
Issue number1
StatePublished - Jan 2014
Externally publishedYes


  • Gave
  • RNA polymerase III
  • Scleroderma renal crisis
  • Systemic sclerosis
  • Watermelon stomach

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology


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