Nongranulomatous ulcerative jejunoileitis (NGUJI) is a rare, often fat
al disorder that produces multiple nonmalignant small bowel ulceration
s. A 55-year-old woman with presumed celiac disease presented with ste
roid-refractory diarrhea, weight loss and abdominal pain. A laparotomy
was performed to exclude the possibility of a lymphomatous disorder,
and multiple nonmalignant small bowel ulcerations were discovered. Des
pite a combination of treatment with total parenteral nutrition (TPN)
and prednisone 30 mg/day she continued to deteriorate. The addition of
azathioprine to her treatment regimen resulted in marked clinical and
biochemical improvement. Her enteroscopy normalized, and she was able
to discontinue TPN and reduce her steroid requirements. Although azat
hioprine has been used occasionally to treat refractory sprue, there h
ave been no reports of its use in NGUJI. In this case, azathioprine pl
ayed a key role in the management of NGUJI and should be considered a
treatment option for patients with this disorder.