Jefferson Institute of Molecular Medicine, Division of Connective Tissue Disease, Department of Dermatology and CutaneousBiology, Division of Rheumatology, Thomas Jefferson University, Philadelphia, Pennsylvania; and Division of Rheumatology, Lehigh ValleyHospital, Allentown, Pennsylvania, USA
OBJECTIVE: We describe 3 patients with systemic sclerosis (SSc) with severe, transfusion-dependent gastric antral vascular ectasia (GAVE) refractory to laser ablation who showed remarkable clinical and endoscopic improvement following intravenous (IV) pulse cyclophosphamide (CYC) treatment. METHODS: Review of clinical records and upper gastrointestinal endoscopy images from 3 patients with SSc and severe GAVE before and after treatment with IV pulse CYC. RESULTS: IV CYC was followed by improvement and stabilization of hemoglobin levels, and marked reduction in blood transfusion requirements and the number and frequency of endoscopic laser treatments. CONCLUSION: IV pulse CYC immunosuppression was followed by remarkable clinical and endoscopic improvement of SSc-associated GAVE.