GASTROINTESTINAL HEMORRHAGE IN PATIENTS WITH SYSTEMIC-SCLEROSIS AND CREST SYNDROME

Objectives: Systemic sclerosis (SSc) and calcinosis, Raynaud's phenome non, esophageal disease, sclerodactyly, telangiectasia (CREST) syndrom e present distinctive microvasculature lesions that are thought to be responsible for tissue damage and disease progression. Involvement of the gastrointestinal tract may lead to the occurrence of profuse hemor rhage. We performed a study to assess the incidence and characteristic s of gastrointestinal hemorrhage in a large group of patients with SSc and CREST syndrome. Methods: We reviewed the medical records of 144 p atients with SSc/CREST seen at our institution during the period 1985- 1996. Endoscopic findings and clinical data were correlated. Data are expressed as means a SD. Results: Twenty-two of 144 (15.2%) patients h ad at least one episode of gastrointestinal hemorrhage (16 women, 4 me n; mean age, 59.4 +/- 17.6 yr). Eight patients (8/22; 36%) had multipl e episodes and four (4/22; 18%) required chronic transfusion therapy. Mucosal telangiectasias were the most common cause of bleeding (9/22; 40.9%), followed by peptic ulcer disease (7/22; 31.8%) and erosive gas tritis (3/22; 13.6%). Bleeding telangiectasias occurred in the entire gastrointestinal tract, including oral cavity (n = 1), esophagus (n = 1), stomach (n = 3), duodenum (n = 1), ileum (n = 1), cecum (n = 2), a nd colon (n = 2). Mortality was 22.7% in patients with gastrointestina l bleeding, compared with 7.3% in patients without bleeding. Conclusio ns: Patients with SSc/CREST syndrome are at risk of developing severe gastrointestinal hemorrhage. This complication is associated with freq uent hospitalization, blood transfusions, and increased mortality. Muc osal telangiectasias are the most common source of bleeding. Appropria te endoscopic intervention is recommended in evaluating and preventing bleeding in patients with SSc/CREST. (Am J Gastroenterol 1998;93: 145 3-1456. (C) 1998 by Am. Cell. of Gastroenterology).