Clinical features of ten cases of cryptogenic multifocal ulcerous stenosing enteritis

Abstract

Objective To investigate the clinical features of cryptogenic multifocal ulcerous stenosing enteritis(CMUSE), and to improve the diagnosis of this rare disease. Methods From 2010 to 2015, clinical data of 10 patients with CMUSE were retrospectively analyzed, including clinical features, laboratory examination, imaging examination, appearance under endoscopy, pathologic characteristics, location of lesions, treatment and prognosis. Results Among the 10 patients with CMUSE (male six, female four), the mean age was (35.1±14.8) years. The predominant clinical manifestation was melena (eight cases), abdominal pain (eight cases) and anemia (nine cases). The results of laboratory examination showed normal in erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Among seven patients, hypersensitive C reactive protein (hsCRP) of two patients increased. Imaging examination showed intestinal stricture and appearance under endoscopic examination was intestinal ulcers and stenosis. Pathologic finding was superficial ulcers at mucosal and submucosal layers. Lesions mostly involved small intestine, and one case involving ileocecal valves and rectum. Among the 10 patients, seven patients received combination of surgical resection and prednisone treatment, and three patients were only administrated with medications. After treatment, seven patients remained remission and three patients relapsed after remission. Immunosuppressors and enteral nutrition was effective in two of them and glucocorticoid resistance happened in one patient. Conclusions The diagnosis of CMUSE should be considered in patients with unexplained recurrent bowel obstruction, melena, anemia and concomitant intestinal ulcer and stricture. Endoscopy plays an important role in the diagnosis. Glucocorticoid is effective but easy to relapse. Immunomodulators and enteral nutrition may be considered as second-line therapy. Key words: Cryptogenic multifocal ulcerous stenosing enteritis; Small intestinal ulcer; Intestinal stenosis; Gastrointestinal hemorrhage; Anemia