The characteristics of intestinal lesions of familial Mediterranean fever gene-related enterocolitis

Abstract

Familial Mediterranean fever (FMF) is a hereditary auto-inflammatory disease in which patients suffer repeated episodes of peritonitis and fever. Generally, the main symptom of FMF is peritonitis, and gastrointestinal mucosal involvement is considered to be rare in FMF patients. Recent reports demonstrated that FMF patients had intestinal lesions mimicking inflammatory bowel disease. However, the information on the intestinal lesions related to FMF has been limited. This review focuses on the possible mechanism of onset of FMF and intestinal lesions in FMF patients, and discusses their endoscopic features. Several reports indicated that the main characteristics of intestinal lesions in FMF patients were ulcerative colitis-like lesions surrounded by reddish mucosa, ulcers and edema with no involvement of the rectum; however, some FMF patients had longitudinal ulcerative lesions and stenosis mimicking Crohns disease. The prevalence of FMF-related enterocolitis among FMF patients remains unclear. Therefore, further accumulation of cases of intestinal lesions in FMF patients will be required for elucidation of their clinical characteristics.