November 26, 2004
Cryptogenic Multifocal Ulcerous Stenosing Enteritis
A patient with anaemia was initially thought to have gastrointestinal cancer and subsequently Crohn's disease, but pathological examination revealed a much rarer condition.
On examination she was pale and unwell. Her abdomen was distended but there was no peritonism. Investigations confirmed iron deficiency anaemia with haemoglobin 9.8 g/dL and ferritin 2 g/L. Her platelet count was also slightly raised at 395 10^sup 9^/L and her erythrocyte sedimentation rate (ESR) was 32 mm/h. Faecal occult bloods were strongly positive. At gastroscopy she was found to have gastroduodenitis but duodenal biopsies were normal. A barium enema revealed no lesions of note. She was initially reassured but her haemoglobin then fell to 9.0 g/dL. Colonoscopy was therefore undertaken but revealed only haemorrhoids and diverticulosis. Colonic and terminal ileal biopsies were normal. Despite oral iron therapy, the haemoglobin fell further to 6.7 g/dL (MCV 56 fL) and her ESR remained raised at 54 mm/h. She was admitted for transfusion and further investigation. Ultrasound revealed 'to-and-fro peristalsis in fluid-filled bowel consistent with intestinal obstruction'. CT confirmed the presence of small-bowel loops and suggested a smoothly tapering stricture in the distal ileum. Small- bowel barium enema showed two smooth strictures in the small intestine with proximal hold-up (Figure 1). At laparotomy two strictures were detected in the proximal ileum and the surgeon felt the appearances were 'typical of Crohn's with fat wrapping and skip lesions'. Histological examination was to prove otherwise. There were three well demarcated ulcerated areas causing separate stenoses. The ulcers were superficial, affecting the mucosa and submucosa, and did not extend to the underlying tissues (Figure 2). A chronic mixed inflammatory cell infiltrate containing some eosinophils was present. The lesions were typical of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE).1 Giant cell granulomas and transmural ulceration were absent, making Crohn's disease unlikely (at a histology meeting none of three pathologists thought the slides consistent with Crohn's), whilst the absence of villous atrophy and malabsorption excluded chronic ulcerative non- granulomatous jejunitis.2 Review of the small-bowel barium study confirmed unusual discrete strictures in the jejunum and proximal ileum which were not typical of Crohn's. Damage induced by non- steroidal anti-inflammatory drugs was offered as an alternative radiological explanation, but apart from an occasional aspirin our patient had not taken such agents for many years.
Figure 1 Small-bowel barium enema showing two discrete smooth strictures with proximal hold-up
Figure 2 Haematoxylin-eosin stain showing inflammation limited to the mucosa and submucosa (double arrow) and not affecting the muscularis propria. The mucosal surface demonstrates a well- demarcated ulcer (block arrow)
Postoperatively the patient's haemoglobin became normal though her ESR remained raised. Vasculitis due to deficiency of complement component C2 has been suggested as a possible cause of CMUSE,3 but in our patient C2 levels were normal. C3 and C4 levels were also normal. Autoantibodies were all negative.
CMUSE, first described in 1964 by Deray et al.,3 is an unusual condition, with fewer than twenty cases reported.4 Most cases present with repeated bouts of small-intestine obstruction. Multiple strictures (2-25) are often present, with frequent relapse following resection. Steroid responsiveness is typical hut steroid dependence often occurs. Limited experience with immunosuppressants (azathioprine and methotrexate) suggests lack of benefit.4 Weight loss was a feature in about half of the cases described and systemic symptoms such as fever and arthralgia may also be present. Iron deficiency anaemia has not, we think, been previously described as the presenting complaint.
1 Fenolio-Preiser CM. Gastrointestinal Pathology: an Atlas and Text, 2nd edn. Philadelphia: Lippincott-Raven, 1999:357
2 Jeffries GH, Steinberg H, Sleisenger MH. Chronic ulcerative (non-granulomatous) jejunitis. Am J Med 1968;44:47-59
3 Debray C, Besancon F, Hardoin JP, et al. Entrite stnosante ulcreuse plurifocale cryptogntique. Arch Fr Mal App Dig 1964;53:195- 206
4 Perlemuter G, Chaussade S, Soubrane G, et al. Multifocal stenosing ulcerations of the small intestine revealing vasculitis associated with C2 deficiency. Gastroenterology 1996;110:1628-32
Hal Spencer MRCP1 Panagiota Kitsanta PhD MD2 Stuart Riley ChB FRCP1
J R Soc Med 2004;97:538-540
Departments of 1 Gastroenterology and 2 Pathology, Northern General Hospital, Herries Road, Sheffield S5 7AU, UK
Correspondence to: Hal Spencer
E-mail: [email protected]
Copyright Royal Society of Medicine Press Ltd. Nov 2004