SUCCESSFUL TREATMENT OF GASTROINTESTINAL VASCULITIS DUE TO SYSTEMIC LUPUS-ERYTHEMATOSUS WITH INTRAVENOUS PULSE CYCLOPHOSPHAMIDE - A CLINICAL CASE-REPORT AND REVIEW OF THE LITERATURE

Gastrointestinal vasculitis in systemic lupus erythematosus (SLE) is q uite rare and almost always accompanied by evidence of active disease in other organs, although occasionally it may be the presenting featur e of the disease. Gastrointestinal involvement in SLE may present as l upus peritonitis, non-necrotizing pancreatitis, gastrointestinal vascu litis or surgical abdomen. Here we report a severe case of SLE which p resented initially with fever of unknown origin. Severe distress, abdo minal pain, the presence of occult blood in the stool and high acute-p hase proteins were explained by a lupus peritonitis and intestinal vas culitis resembling inflammatory bowel disease. Whereas high-dose predn isone treatment did not prevent a severe relapse, we observed a sustai ned remission following i.v. cyclophosphamide pulse therapy. In the li terature, only two similar cases are reported: one died despite a chan ge in the therapy of a bowel perforation; our case was the second that improved under pulse cyclophosphamide. We suggest the use of cyclopho sphamide after failure of steroids early in the course of SLE gastroin testinal vasculitis to prevent devastating complications.