Gastric antral vascular ectasia in systemic sclerosis: Where do we stand?

Abstract

Gastric antral vascular ectasia (GAVE) continues to be a challenge in both diagnosis and treatment. GAVE has a diverse group of associations and presumed causes, including cirrhosis, chronic renal failure and autoimmune connective tissue diseases. However, in most occasions, the management plan of GAVE itself is the same whatever the underlying disease by using Argon plasma coagulation (APC). Herein, we will discuss three cases of systemic sclerosis-associated GAVE presenting with either acute or chronic gastrointestinal bleeding showing variable responses to APC. Anemia and telangiectasia may be the first striking presentation of systemic sclerosis (SSc). Renal artery stenosis, aortic stenosis, widespread cutaneous and mucosal telangiectasia and hypertension seem to be associated with poor prognosis and should prompt rapid intervention and careful follow-up. Moreover, the hunt for molecular underpinnings of the broad array of vascular lesions in SSc has to include von Willebrand factor and endoglin. Eventually, we will review the recent alternatives that can be effective in SSc-GAVE, such as band ligation, hematopoietic stem cells transplantation and immunotherapy.

Radiofrequency ablation (RFA) vs. argon plasma coagulation (APC) for the management of gastric antral vascular ectasia (GAVE) in patients with and without cirrhosis: results from a retrospective analysis of a large cohort of patients treated at a single center

“…Despite this association between GAVE and cirrhosis, bleeding from GAVE does not respond to therapy directed at reducing portal blood pressure, suggesting that it may be an immunologic or hormonal phenomenon 8 . GAVE is also associated with chronic kidney disease 1 9 , heart disease 1 10 and autoimmune connective tissue diseases such as systemic sclerosis 9 11 .…”

HSCT-GAVE as a Manifestation of Chronic Graft versus Host Disease: A Case Report and Review of the Existing Literature

“…Autoantibodies are an expression of B-cell hyperactivity promoted by autoreactive T-cells in autoimmune diseases and by donor T-cells in chronic GVHD [ 19 ]. Autoantibodies may cross-react with specific proteins in the gastric mucosa and submucosa leading to inflammation, vascular endothelial cell injury, and ultimately the local release of factors resulting in thrombus formation in these small vessels [ 19 , 20 ]. Additionally, GAVE in scleroderma patients (SSc-GAVE) has been associated with specific antibodies.…”

“…Additionally, GAVE in scleroderma patients (SSc-GAVE) has been associated with specific antibodies. One study demonstrated that patients with SSc-GAVE were positive for anti-RNA polymerase III at a rate of 50% compared to 16% in patients with scleroderma and no evidence of GAVE [ 20 , 21 ]. GAVE lesions in scleroderma have been reported to resolve with immunosuppressive therapy, and gastric biopsies from cases of SSc-GAVE also show CD4+ T-cell infiltration [ 19 , 22 , 23 ].…”

Haematological abnormalities in systemic sclerosis

“…According to literature data, the most common cause of erythrocytic system pathology in systemic scle-rosis patients is microcytic anaemia associated with iron deficiency and resulting from relatively common gastrointestinal microhaemorrhages and gastric antral vascular ectasia (GAVE), also known as watermelon stomach, endoscopically characterised by red stripes in the lining [3][4][5].…”