Presentation: Gastric Antral Vascular Ectasia in Systemic Sclerosis (2007)

15 Gastric Antral Vascular Ectasia in Systemic Sclerosis

Purpose: Gastric Antral Vascular Ectasia (GAVE, watermelon stomach) is a rare form of upper gastrointestinal blood loss that is often clinically silent until severe anemia is present. The association of GAVE and autoimmune disease has been described. We evaluated a series of patients with both systemic sclerosis (SSc) and GAVE to further characterize this disease association and to make predictions about which SSc patients are at risk for GAVE.
Methods: Twenty-five patients with both SSc and GAVE were identified from two university hospitals. A retrospective chart review was conducted to obtain the following data: demographics, SSc subtype, antibody status, timing of symptom onset and diagnoses, GAVE treatment, medication use, SSc disease manifestations, and underlying medical conditions.
Results: Twenty-three patients were female and 23 were Caucasian (no African Americans). GAVE preceded the onset of SSc in 1 patient, and it followed the onset in the other 24 patients. Twelve of the 15 diffuse cutaneous SSc (dcSSc) patients had rapid progression of skin thickening early in the disease, and only one of these patients was anti-topoisomerase I positive. In the 10 limited cutaneous SSc (lcSSc) patients, 7 had anticentromere antibody. GAVE was diagnosed closer to the onset of SSc symptoms in the dcSSc group (mean 22.3 months, SD 28.5) when compared to the lcSSc group (mean 79.8 months, SD 88.4) (p=0.027). The overall mean nadir hematocrit was 25.7% (18%-43%), and the group as a whole required 4.6 (0-40) blood transfusions and 3.6 (0-16) endoscopic laser treatments. The dcSSc group had a trend toward lower nadir hematocrit and required more transfusions and laser treatments than the lcSSc group, but this was not statistically significant. Only 7 patients were not on proton pump inhibitors (PPIs) at the onset of GAVE. Other vascular findings including telangiectasias (15), essential hypertension (14), pulmonary hypertension (5), and renal crisis (4) were seen in SSc patients with GAVE.
Conclusions: This represents the largest series of GAVE in SSc. Interestingly, no African American patients were identified despite both centers having a 20% frequency of African Americans in their SSc population. The patients fell predominantly into two groups: anti-topoisomerase I negative dcSSc with rapid progression of skin thickening early in the disease, and anticentromere positive lcSSc. DcSSc patients were more likely to develop GAVE closer to the onset of SSc symptoms compared to the lcSSc patients. Nearly 75% of the patients were on PPIs at the onset of GAVE, and it is interesting that prolonged use of these medications has been linked to the development of atrophic gastritis, another disease associated with GAVE. New anemia, particularly in early dcSSc patients, should prompt the clinician to carefully evaluate for GAVE.

 K.M. Ingraham, None; M.S. O'Brien, None; C.T. Derk, None; V.D. Steen, None.