Background/AimsGastrointestinal involvement in vasculitis may result in life-threatening complications. However, its variable clinical presentations and endoscopic features, and the rarity of the disease, often result in delayed diagnosis.Methods : Clinical characteristics, endoscopic features, and histopathological findings were reviewed from medical records.Results : Of 6,477 patients with vasculitis, 148 were diagnosed as primary vasculitis with upper gastrointestinal involvement. Of these, 21 cases (14.2%) were classified as large-vessel vasculitis, 17 cases (11.5%) as medium-vessel vasculitis, and 110 cases (74.3%) as small-vessel vasculitis. According to the specific diagnosis, IgA vasculitis (Henoch-Schönlein purpura) was the most common diagnosis (56.8%), followed by Takayasu arteritis (14.1%), microscopic polyangiitis (10.1%), and polyarteritis nodosa (6.8%). Gastrointestinal symptoms were present in 113 subjects (76.4%), with abdominal pain (78.8%) the most common symptom. Erosion and ulcers were striking endoscopic features, and the second portion of the duodenum was the most frequently involved site. Biopsy specimens were obtained from 124 patients, and only eight (5.4%) presented histopathological signs of vasculitis.Conclusion : sDiagnosis of vasculitis involving the upper gastrointestinal tract is difficult. Because of the widespread use of endoscopy, combining clinical features with endoscopic findings may facilitate making appropriate diagnoses; however, the diagnostic yield of endoscopic biopsy is low.