TY - JOUR
T1 - Prevalence, correlates and outcomes of gastric antral vascular ectasia in systemic sclerosis
T2 - A eustar case-control study
AU - Ghrénassia, Etienne
AU - Avouac, Jérome
AU - Khanna, Dinesh
AU - Derk, Chris T.
AU - Distler, Oliver
AU - Suliman, Yossra Atef
AU - Airo, Paolo
AU - Carreira, Patricia E.
AU - Foti, Rosario
AU - Granel, Brigitte
AU - Berezne, Alice
AU - Cabane, Jean
AU - Ingegnoli, Francesca
AU - Rosato, Edoardo
AU - Caramaschi, Paola
AU - Hesselstrand, Roger
AU - Walker, Ulrich A.
AU - Alegre-Sancho, Juan Jose
AU - Zarrouk, Virginie
AU - Agard, Christian
AU - Riccieri, Valeria
AU - Schiopu, Elena
AU - Gladue, Heather
AU - Steen, Virginia D.
AU - Allanore, Yannick
PY - 2014/1
Y1 - 2014/1
N2 - 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
AB - 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
KW - Gave
KW - RNA polymerase III
KW - Scleroderma renal crisis
KW - Systemic sclerosis
KW - Watermelon stomach
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U2 - 10.3899/jrheum.130386
DO - 10.3899/jrheum.130386
M3 - Article
C2 - 24293584
AN - SCOPUS:84891812975
SN - 0315-162X
VL - 41
SP - 99
EP - 105
JO - Journal of Rheumatology
JF - Journal of Rheumatology
IS - 1
ER -