Isolated Superior Mesenteric Artery Vasculitis with Response to Glucocorticoids

This report describes a patient with abdominal pain at presentation, which on angiography was found to be caused by vasculitis limited to the superior mesenteric artery. He was treated with oral glucocorticoids after an initial 5-day course of pulse therapy and obtained excellent relief of symptoms. Steroids were tapered over 8 months. Repeat angiography revealed complete resolution of the vasculitis with a residual aneurysm of a branch of the left gastric artery, which was resected to avoid the risk of rupture. Histologic analysis revealed thickened intima with myxoid degeneration and no evidence of active vasculitis. Extensive laboratory and clinical investigations did not reveal an underlying connective tissue disorder to account for the gastrointestinal vasculitis. This report of an angiographically defined regional vasculitis involving the superior mesenteric artery with complete clinical as well as radiologic resolution on treatment with glucocorticoids alone emphasizes the need to differentiate between an isolated or regional vasculitis, which has an excellent prognosis, to avoid subjecting the patient to cytotoxic therapy with significant toxicities.