Ulcerative Jejunoileitis

Definition

• Chronic idiopathic ulcerative process of the small intestine associated with celiac disease and enteropathy-type T cell lymphoma

Alternate / Historic names

• Chronic ulcerative jejunitis
• Non-granulomatous ulcerative jejunitis
• Ulcerative enteritis
• Ulcerative jejunitis

Diagnostic Criteria

• Multiple ulcers in jejunum and/or ileum
• Usually transversely oriented
• Variable depth
• May perforate
• Mixed inflammatory infiltrate in ulcers
• Granulomas not seen
• Scarring may lead to strictures
• Villous atrophy of surrounding mucosa
• Usually arises in the setting of refractory celiac disease or villous atrophy of unknown type
• Diagnosis of celiac disease frequently made concurrently with the ulcers
• May represent a precursor or early stage of enteropathy type T cell lymphoma
• Most cases have a clonal T cell population
• Subsequent lymphomas express the same T cell receptor gene rearrangement as seen in the ulcerative jejunoileitis

Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting : November 11, 2009

Differential Diagnosis

• Infectious processes must be ruled out by stool examination and culture and histology

Crohn Disease	Ulcerative Jejunoileitis
Frequently also involves large intestine	Restricted to small intestine
Adjacent mucosa normal	Villous blunting of adjacent mucosa
May have granulomas	No granulomas

• Clonal T cell populations are present in both
• Some believe that ulcerative jejunoileitis is an early form of enteropathy-type T cell lymphoma

Clinical

• Presents with abdominal pain, diarrhea, bleeding
• May present with perforation
• High mortality
• Bowel obstruction
• Perforation
• Development of overt lymphoma

Bibliography

• Noffsinger A, Fenoglio-Preiser CM, Maru D, Gilinisky N.  Gastrointestinal Diseases, AFIP Atlas of Nontumor Pathology, First Series, Fascicle 5, 2007
• Ashton-Key M, Diss TC, Pan L, Du MQ, Isaacson PG. Molecular analysis of T-cell clonality in ulcerative jejunitis and enteropathy-associated T-cell lymphoma. Am J Pathol. 1997 Aug;151(2):493-8.
• Biagi F, Lorenzini P, Corazza GR. Literature review on the clinical relationship between ulcerative jejunoileitis, coeliac disease, and enteropathy-associated T-cell. Scand J Gastroenterol. 2000 Aug;35(8):785-90.
• Cellier C, Delabesse E, Helmer C, Patey N, Matuchansky C, Jabri B, Macintyre E, Cerf-Bensussan N, Brousse N. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet. 2000 Jul 15;356(9225):203-8.