P53 Gastric antral vascular ectasia in Sjögren's syndrome?

Abstract

Abstract Background Gastric antral vascular ectasia (GAVE) is a rare, but severe cause of non-variceal upper gastrointestinal bleeding (UGIB). It commonly manifests as iron deficiency bleeding and associated with liver cirrhosis, chronic renal failure and connective tissue diseases. Many therapeutic options have been proposed, but the first line of treatment for GAVE remains as endoscopic Argon plasma coagulation (APC). At present, there are no clear satisfactory results to opt for medical therapy as an alternative to invasive approach. We present a case of primary Sjögren's syndrome who has developed GAVE and successfully treated with medical therapy. Methods A 68 years old lady was initially referred to Rheumatology with new symptoms of Raynaud’s, affecting her hands and toes. She had dry eyes and mouth, which were managed conservatively. There was no skin change or gastrointestinal symptoms. She had strongly positive ANA with negative ENA and low levels of IgG and IgM. Nifedipine was initiated to control her Raynaud’s symptoms and the diagnosis of Sjogren’s syndrome was made. Eight months following the initial diagnosis, she was found to have iron deficiency anaemia. There was no change in her symptoms or dietary intake. CT colonography revealed no colonic abnormality. Gastroscopy revealed gastric antral vascular ectasia (GAVE) and the patient was treated with endoscopic argon plasma coagulation (APC). This patient underwent second APC after three months as her haemoglobin dropped despite regular iron supplements. We had made a decision to start pulsed cyclophosphamide and methylprednisolone infusion to control her disease activity. Her haemoglobin remained stable for seven months following the last infusion. She then underwent her third APC and another pulsed cyclophosphamide infusion was resumed. Nine months since her last infusion, her haemoglobin dropped once again, and she underwent another APC. In view of her good response to cyclophosphamide infusion, this lady was started on mycophenolate as maintenance. Since the introduction of mycophenolate, we were pleased that this lady’s haemoglobin had normalised, and the iron supplement was no longer required. Results The association of GAVE with Sjögren's syndrome is not commonly known. There were two other case reports of patients with primary Sjögren's syndrome and developed a mild form of GAVE. As patients with GAVE in the absence of cirrhosis are more at risk of active upper gastrointestinal bleeding, they require more frequent endoscopic treatments following initial diagnosis. APC is an effective treatment; however, it is uncomfortable for these patients to experience repeatedly. Conclusion This case has shown the importance of recognition the possible association of primary Sjögren's and GAVE syndrome as a cause of iron deficiency anaemia. The successful medical therapy of GAVE provides a safe alternative for endoscopic APC, however, more evidences are required to prove the significant of the outcome. Disclosures Y. Tan None. A. Gough None.