GAVE in Systemic Sclerosis: A Japanese Cohort Study Highlighting Links to Diffuse Cutaneous Subtype and Anti-RNAPIII Antibodies.

Gastric antral vascular ectasia (GAVE) is a cause of gastrointestinal bleeding in systemic sclerosis (SSc), but its predictors and outcomes in SSc are not fully defined. We aimed to determine the frequency of GAVE in SSc and to compare the clinical characteristics of SSc patients with versus without GAVE in a single-center Japanese cohort. We retrospectively identified SSc patients who underwent upper gastrointestinal endoscopy at our institution between 2006 and 2015. Clinical data including SSc subtype, extent of skin sclerosis, and autoantibody profiles were collected for patients with endoscopically confirmed GAVE and for those without GAVE. Of 272 SSc patients who underwent endoscopy, 19 patients (7.0%) had GAVE. SSc patients with GAVE were significantly more likely to have diffuse cutaneous SSc and had a shorter disease duration since SSc onset compared to those without GAVE. GAVE patients tended to have more severe skin sclerosis and a markedly higher prevalence of anti-RNA polymerase III antibody (42% vs. 9.5% in non-GAVE, p < 0.001), with a corresponding lower frequency of anti-centromere antibody (10.5% vs. 45.4%, p < 0.001). Scleroderma renal crisis occurred in 16% of GAVE patients versus < 1% of non-GAVE patients (p < 0.001). Follow-up endoscopy in GAVE patients showed that in roughly half of the cases the GAVE lesions disappeared over 1-8 years (often after immunosuppressive therapy), whereas the remainder had persistent GAVE changes. GAVE occurred in about 7% of SSc patients, especially those with diffuse, rapidly progressive disease. It was associated with anti-RNA polymerase III positivity and early severe organ involvement. Patients with early diffuse skin disease and this antibody may be at higher risk, highlighting the importance of early detection to prevent bleeding and anemia.