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Gastric Antral Vascular Ectasia (GAVE) in Patients with Systemic Sclerosis (SSc): a Systematic Review of the Literature

Posted Oct 09 2009 10:04pm
Purpose:

Gastric antral vascular ectasia (GAVE) or “watermelon stomach” is a rare but important cause of iron-deficiency anemia that is often associated with autoimmune or connective-tissue disorders. It has been described in conjunction with systemic sclerosis (SSc), but a comprehensive and up-to-date review of the literature has not been performed. A systematic review of the literature was conducted in order to describe the clinical parameters, typical presentation, preferred treatments, and outcomes for SSc-associated GAVE (SSc-GAVE).

Methods:

The medical literature was interrogated using Ovid MEDLINE, CINAHL (Cumulative Index to Nursing & Allied Health Literature), and U.S. National Library of Medicine’s PubMed services with the terms watermelon stomach, gastric antral vascular ectasia, systemic sclerosis, and scleroderma. In addition, all previous issues of Scleroderma Care and Research were manually searched, and references from retrieved papers were reviewed. This search yielded a total of 22 articles that met the pre-specified criteria (a detailed case report, translatable language, and patients diagnosed with both GAVE and SSc), producing 56 detailed case reports (including 8 cases derived from our own database). Data were systematically compiled and analyzed using Microsoft Excel 2003.

Results:

The median age of patients with GAVE and SSc was 60.1 years, with 86% female patients. The subtypes of SSc were 50% limited and 50% diffuse. The average duration between the diagnosis of SSc and the subsequent diagnosis of GAVE was 1.6 years. ANA was positive in 77% of the cases. Anemia was the presenting symptom of GAVE in 94% of the cases with 23% reporting melena. Pernicious anemia complicated 19% of cases. Eighty-six percent of the patients required transfusions, with a median duration of 12.0 months during which time the patients were bleeding (mean number of transfused units = 14.5 per patient). Over 50% of patients were treated by Nd:YAG laser, 20% percent required surgery, and 8% remained transfusion dependent. Patients required surgical or endoscopic re-treatment in 36% of cases.

Conclusions:

Although GAVE is a rare clinical entity, it should be considered in patients with SSc with iron-deficiency anemia. GAVE affects people with limited and diffuse SSc in equal proportions. Given that limited scleroderma is more common than diffuse scleroderma in most unselected cohorts, this apparently equal distribution may in fact suggest that GAVE disproportionately affects patients with diffuse disease.

SSc-GAVE patients often require continued laboratory monitoring and frequent transfusions of significant volume. Consideration should be given to prophylactic erythropoiesis-stimulating agents and Nd:YAG laser. Patients may require re-treatment, continued medical management, or even surgery to control the associated anemia.

L. Caplan, None.
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