Numerous diseases cause small intestinal ulcers (Table 6.9). The rare disorder, chronic ulcerative jejunitis, also known as nongranulomatous chronic idiopathic enterocolitis and chronic ulcerative nongranulomatous jejunoileitis (251), is usually rapidly fatal and has a controversial etiology. Patients are typically middle-aged individuals who present with abdominal pain, anorexia, weight loss, fever, diarrhea,
malabsorption, steatorrhea, hypoalbuminemia, protein-losing enteropathy, celiac disease, lymphoma, hypogammaglobulinemia, pulmonary fibrosis, and polymyositis. Features shared with celiac disease include generalized severe malabsorption, profuse diarrhea, villous atrophy, and an intense mucosal mononuclear infiltrate, but dietary gluten exclusion does not ensure clinical improvement because the changes typically occur in refractory sprue. The clinical features mimic those of Crohn disease due to the presence of granulomas in some biopsies and a clinical response to anti-inflammatory drugs in some patients.