Localized vasculitis of the gastrointestinal tract: a case series.

OBJECTIVE To describe the clinical features and outcomes of patients with localized vasculitis of the gastrointestinal tract (LVGT). METHODS Medical records of 608 patients diagnosed with vasculitis involving the intra-abdominal vasculature and/or abdominal viscera between January 1996 and December 2007 were reviewed. Only patients with histopathological confirmation or typical angiographic findings of vasculitis localized to the abdomen were included. RESULTS We identified 18 cases with LVGT over the 12-year study period. The patients were predominantly Caucasian (89%) and female (67%) with a median age at diagnosis of 53.5 (range 17.4-83.3) years. Most of the patients presented with abdominal pain and 12 (66.6%) patients presented with an acute abdomen requiring surgical intervention. At diagnosis, the median ESR was 30.5 (range 4-77) mm/h. Autoantibody screening was generally unrevealing. Abdominal CT scan findings included: bowel wall thickening, bowel infarction and solid organ infarcts. In 14 patients, the diagnosis of vasculitis was established by abdominal angiography. Histological evidence of vasculitis was recorded in 5 (28%) patients, most commonly from gall bladder or small intestine specimens. Corticosteroid therapy was administered to 10 (56%) patients, 5 of whom also received other immunosuppressive agents. Median duration of follow-up was 10.5 (range 2-156) months. No evidence of vasculitis outside the abdomen was observed during follow-up. Seven (39%) patients died during the follow-up period. Survival of the patient cohort (compared with an age-matched US white population) was significantly reduced (P < 0.001). CONCLUSION LVGT is an uncommon form of vasculitis that can be associated with significant morbidity and mortality.