—  SHORT COURSE #44  —

Non-Neoplastic Disorders of the Intestines

Case 10 - Duodenal Involvement By Ulcerative Colitis

Laura W. Lamps, M.D.
Audrey J. Lazenby, M.D.
Joel K. Greenson, M.D.


Clinical Presentation:
A 20 year old female underwent a subtotal colectomy with ileostomy for ulcerative colitis three years ago. Nine months after surgery, the patient became symptomatic again, prompting evaluation.


Case 10 - Figure 1 - Low magnification shows moderate blunting of duodenal villi and expansion of the lamina propria by a lymphoplasmacytic infiltrate.

Case 10 - Figure 2 - Higher magnification emphasizes architectural distortion of the duodenal glands, and the dense lymphoplasmacytic infiltrate.

Case 10 - Figure 3 - Focal cryptitis, crypt abscesses, and gland loss are noted.


Endoscopic Findings:
At endoscopy, the duodenum showed "diffuse enteritis."

Diagnosis :
Duodenal involvement by ulcerative colitis

General Comments:
Traditionally, ulcerative colitis (UC) and Crohn's disease (CD) have been distinguished clinically and histologically by anatomic distribution as well as by patterns of inflammation associated with them. CD is classically regarded as a discontinuous disease with skip areas, whereas UC is characterized as a diffuse mucosal disease of the colon with continuous and symmetric involvement, virtually always involving the rectum. Other than "backwash ileitis," UC has traditionally been regarded as sparing the small bowel completely.

Several recent publications have challenged these traditional views regarding the anatomic distribution of UC. Several studies based on colorectal biopsies from chronic UC patients have demonstrated rectal sparing, patchy rather than diffuse colitis, and even complete rectal healing during the course of chronic disease. Rare case reports of patients with histologically documented UC associated with small intestinal involvement also appear in the literature, further challenging the classic dogma relating to the anatomic distribution of UC.

Diffuse duodenitis has now been well described in patients with confirmed diagnoses of UC. Duodenal involvement is often diagnosed when there is persistent nausea, vomiting, and/or bloody diarrhea in patients who have already had their colons resected for UC.

Pathologic features:
Endoscopically, findings are similar to colonic UC with diffusely erythematous, friable mucosa. Histologically, the features are similar to UC within the large bowel, showing diffuse mucosal inflammation with basal plasmacytosis of the lamina propria, neutrophilic cryptitis, crypt abscesses, and mucosal crypt distortion.

Differential diagnosis:
The major item in the differential diagnosis, of course, is CD. No other clinical, radiographic, or endoscopic features of CD should be present, nor should gross or microscopic findings of CD exist in either the duodenal biopsy in question or in previous specimens. The recognition of duodenal involvement by UC suggests that rather than automatically diagnosing CD in all patients presenting with pancolitis and diffuse duodenitis, one should consider the possibility of UC with an aberrant anatomic distribution as these patients may be candidates for successful re-anastomosis or ERPT procedures.

It remains to be determined whether duodenal involvement by UC is a previously unrecognized complication of chronic UC, a component of a variant type of UC, or possibly a completely different concurrent disease entity. Although this type of small intestinal involvement is not typical of the natural history of UC, it is possible that it is a rare complication of UC. As traditional views regarding the distribution of UC are already changing, further studies are needed to better understand and characterize the issue of upper small intestinal inflammation in UC.

Treatment:
Cases of duodenal involvement by UC in the literature have done well treated with medical therapy for UC.

References:
  1. Bernstein CN, Shanahan F, Anton P, Weinstein W. Patchiness of mucosal inflammation in treated ulcerative colitis: a prospective study. Gastrointest Endoscopy 42:232-7, 1995.

  2. D'Haens G, Geboes K, Peeters M, Baert F, Ectors N, Rutgeerts P. Patchy cecal inflammation associated with distal ulcerative colitis: a prospective endoscopic study. The American Journal of Gastroenterology 8:1275-9, 1997.

  3. Kleer CG, Appelman HD. Ulcerative colitis: Patterns of involvement in colorectal biopsies and changes with time. Am J of Surg Pathol 22:983-989, 1998.

  4. Mitomi H, Atari E, Uesugi H, et al. Distinctive diffuse duodenitis associated with ulcerative colitis. Digestive Disease and Sciences 42:684-93, 1997.

  5. Odze R, Antonioli D, Peppercorn M, Goldman H. Effect of topical 5-aminosalicylic acid (5-ASA) treatment on rectal mucosal biopsy morphology in chronic ulcerative colitis. Am J Surg Pathol 17:869-75, 1993.

  6. Valdez R, Appelman HD, Bronner MP, Greenson JK. Diffuse duodenitis associated with ulcerative colitis. Am J Surg Pathol 24:1407-13, 2000.

  7. Sasaki M, Okada K, Koyama S, et al. Ulcerative colitis complicated by gastroduodenal lesions. J Gastroenterology 31:585-89, 1996.