Case report of cryptogenic multifocal ulcerous stenosing enteritis (CMUSE): a rare disease may contribute to endoscopy-capsule retention in the small intestine
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CMUSE is a rare disease whose diagnosis remains difficult because the lesion is confined to the small bowel.
Here, we present a case of 43-year-old female patient suffered chronic abdominal pain for 20 years, and finally diagnosed with CMUSE. Capsule endoscopy was performed when general endoscopic investigation failed to find the lesion, but the capsule was stranded in the small intestine. Moreover, capsule retention results in acute intestinal obstruction. Thus, surgery was performed and CMUSE was confirmed. The patient was recovered after partial small intestine resection.
Capsule retention occurred in nearly 60% of patients with CMUSE. Capsule endoscopy should be avoided when the patient is suspected of CMUSE, especially with severe anemia and radiologic finding in the ileum.
KeywordsCryptogenic multifocal ulcerous stenosing enteritis Capsule retention Diagnosis Anemia
Chronic enteropathy associated with SLCO2A1 gene
Cryptogenic multifocal ulcerous stenosing enteritis
Chronic nonspecific multiple ulcers
Double balloon endoscopy
Neuromuscular and vascular hamartoma
Small bowel series
Cryptogenic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease with unknown etiology and pathophysiology. This disease is an independent entity characterized by chronic and intermittent bouts of moderate ileus resulting from multiple short stenoses of the small bowel with shallow ulcers [1, 2, 3]. It has been reported in both adults and children but were mainly diagnosed during adulthood with a mean age of approximate 40 years [2, 4, 5, 6]. The clinical characteristics of CMUSE include abdominal pain and iron-deficiency anemia, moreover, the location of ulcerative strictures was usually located in the ileum [4, 5, 7]. The diagnosis of CMUSE remains difficult because of the unspecific clinical manifestations and vague radiologic findings such as the presence or absence of strictures and superficial ulcers in abdominal computed tomography (CT) or small bowel series (SBS). Thus, small intestine wireless capsule endoscopy (CE) seems to be the best non-invasion inspection of CMUSE. However, capsule retention in the bowel is becoming a common complication of CMUSE should not be neglected.
A 43-year-old woman with remittent abdominal pain, dizziness, and fatigue for 20 years was admitted to our hospital in May 2018. She was also suffered from iron deficiency anemia since her adolescence. There were positive results of stool occult blood test but no evidence of bleeding in gastroscopy and colonoscopy during the progression of the disease. On examination, she had lower limbers edema and mild abdominal tenderness around the umbilicus. Vital signs were within normal range.
Discussion and conclusions
Since Debray et al.  reported the first case of CMUSE in 1964, this rare disease has been found in the worldwide region. and Matsumoto et al.  call this distinctive entity chronic nonspecific multiple ulcers (CNSU) of the small intestine. However, CNSU is now considered as a different entity called chronic enteropathy associated with SLCO2A1 gene (CEAS) . Moreover, neuromuscular and vascular hamartoma (NMVH) is supposed to be the same disease of CMUSE . The etiology of CMUSE is still unclear, but several hypotheses such as immunopathological pathogenesis , excessive formation of fibrous tissue and disturbance of collagen degradation [3, 11], vasculopathy induced by partial C2 deficiency [2, 12], recessive mutations in the PLA2G4A  genes had been suspected.
CMUSE is a chronic and recurrent disease. The most common symptoms include abdominal pain, anemia, and gastrointestinal bleeding [4, 5]. Diagnosis of this disease remains difficult due to the lack of specific characteristics and the lesion mainly located in the small bowel. Differential diagnosis of CMUSE includes Crohn’s disease (CD) [14, 15], NSAIDs-induced enteropathy , tuberculous enteritis and other chronic infections of the small intestine [17, 18, 19].
In order to directly observe the lesions in the small intestine, both CE and DBE are viable [20, 21, 22]. Generally, capsule endoscopy, serve as non-invasive detection, is preferred to use before balloon endoscopy. However, the capsule retention rate of CMUSE patients is extremely high which should not be neglected. After reviewing pieces of literature of CMUSE and some cases in China, 22 patients with CMUSE were performed CE and the presence of capsule retention is also clearly described. Surprisingly, 13 patients underwent capsule retention [3, 5, 10, 14, 17, 23, 24, 25, 26, 27, 28, 29, 30]. It means that nearly 60% of patients with CMUSE who receives CE will contribute to retention in the small intestine. Capsule retention has been reported in approximately 1.4% of CE procedures . Even in the patient with known CD, the incidence rate is only about 13% .
Clinical characteristics of the patients diagnosed with CMUSE
As for treatment, the most effective is immunotherapy [2, 3] and surgery. However, the recurrence rate of CMUSE is high [2, 5], and most patients develop corticosteroid dependence. Intriguingly, infliximab (anti-TNF-α therapy) was reported useful in CMUSE . Moreover, gene mutations are closely related to this disease , genetic therapy may be effective for this rare disease in the future.
Availability of data and materials
All the data supporting the conclusions of this article are included within the published article and its additional files.
THZ, YFW, JTT, YXC, and QYG carried out the clinical diagnosis, provided the clinical details and participated in designing the report. EWT and QYG drafted the manuscript. All authors have read and approved the final manuscript.
Ethics approval and consent to participate
Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor in Chief of this journal.
The authors declare that they have no competing interests.
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- 8.Matsumoto T, Iida M, Matsui T, Yao T. Chronic nonspecific multiple ulcers of the small intestine: a proposal of the entity from Japanese gastroenterologists to Western enteroscopists. Gastrointest Endosc 2007;66(3 Suppl):S99–107.Google Scholar
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