title: Nongranulomatous chronic idiopathic enterocolitis: clinicopathologic profile and response to corticosteroids.
abstract: OBJECTIVE
  Nongranulomatous ulcerative enterocolitis has been reported in association with celiac sprue, lymphoma, and hypogammaglobulinemia. The objective of this study is to present evidence that this disorder exists as a primary entity.
  
  
  METHODS
  The medical records and histological material of 9 patients (mean age, 45.7 +/- 5.9 years) who presented with severe chronic diarrhea without specific diagnosis after extensive investigations were reviewed.
  
  
  RESULTS
  Endoscopically, the duodenum and proximal jejunum were inflamed in 6 of 7 patients, with superficial ulcerations in 5 patients. On histology, the lamina propria was infiltrated by polymorphonuclear and chronic inflammatory cells, with varying degrees of villous atrophy. There were no significant cellular abnormalities of the epithelial enterocytes. A similar inflammatory infiltrate was present in the colon in 4 or 5 patients. Eight of 9 patients responded to corticosteroids with clinical and variable histological improvement. Four patients developed bleeding from ulcerations in the small or large intestine. Three patients died: 1 patient who did not respond to treatment with corticosteroids and 2 patients with systemic infection. Four of the 6 surviving patients required maintenance low-dose corticosteroid therapy. No underlying disease was discovered during prolonged follow-up.
  
  
  CONCLUSIONS
  Idiopathic nongranulomatous enterocolitis may present as a primary, frequently fatal disease. Corticosteroid therapy provides immediate benefit and may be required indefinitely.
authors.affiliation: Department of Medicine, Medical College of Wisconsin, Milwaukee, USA.
authors.forename: E A
authors.initials: EA
authors.lastname: Ruan
authors.forename: R A
authors.initials: RA
authors.lastname: Komorowski
authors.forename: W J
authors.initials: WJ
authors.lastname: Hogan
authors.forename: K H
authors.initials: KH
authors.lastname: Soergel
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D001706:Biopsy; D002908:Chronic Disease; D004760:Enterocolitis / Q000188:drug therapy* / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007422:Intestines / Q000473:pathology; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones