INTESTINE Vol.23 No.6(4-3)

Theme Systemic disease-associated intestinal involvement -- Intestinal Behçet's disease and its differential diagnosis
Title FMF-related enterocolitis
Publish Date 2019/11
Author Hiroshi Nakase Department of Gastroenterology and Hepatology, Sapporo Medical University School of Medicine
[ Summary ] Familial Mediterranean fever (FMF) is a hereditary auto-inflammatory disease with characteristics of repeated peritonitis and fever. Recent reports demonstrated that FMF patients had intestinal lesions mimicking inflammatory bowel disease. This review focuses on the possible mechanism of FMF onset and intestinal lesions in FMF patients and discusses the endoscopic features. Our current data suggest that UC-like lesions such as surrounding reddish mucosa, erosions, ulcers, and edema were mainly observed in FMF patients with no involvement of the rectum, while the longitudinal ulcerative lesions and stenosis mimicking Crohn's disease were observed. The prevalence of patients with FMF related enterocolitis remains unclear. Therefore, further accumulation of cases of the intestinal lesions in FMF patients will be required for elucidation of their clinical characteristics.
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