Familial Mediterranean fever with sigmoid colon stricture

Abstract

We describe a case of familial Mediterranean fever (FMF) with sigmoid colon stricture. The patient, a woman in her 30 s, had a 12-year history of ileocolitis-type Crohn's disease. The colonoscope could not pass because of the sigmoid colon stricture, and the patient was referred to our hospital with complaints of abdominal pain and fever. At 2-month postreferral, the patient presented with severe abdominal pain and fever. Computed tomography and intestinal ultrasonography revealed no bowel obstruction, whereas wall thickening was observed in the sigmoid colon and small bowel. Our medical interview revealed a cyclical nature to the symptoms. We diagnosed FMF and initiated colchicine. Subsequently, for more than 2 years, the patient remained asymptomatic, and the sigmoid colon stricture improved. FMF should be considered in patients with inflammatory bowel disease with periodic abdominal pain and fever.

Keywords: Colchicine; Crohn’s disease; Familial Mediterranean fever; Inflammatory bowel disease; Intestinal stricture.

Fig. 1

Image of the colonoscopy performed 3 years before her visit to the hospital while on 5-aminosalicylic acid and azathioprine treatment. a Cecum. b Ascending colon. c Transverse colon. d Descending colon. From the cecum to the descending colon, the findings were normal colon mucosa. e Sigmoid colon. Scattered longitudinal erosions were observed. f Rectum. Erythema and aphthae were detected

Fig. 2

Sigmoid colon 2 months before the hospital visit. Stricture with ulceration prevented the passage of the colonoscope

Fig. 3

Computed tomography image at the time of exacerbation. Intestinal wall thickening with contrast effect was observed in the sigmoid colon and small intestine. The jejunum and sigmoid colon are indicated by the yellow and green arrowheads, respectively. a Horizontal section at the level of the jejunum. b Horizontal section at the level of the Sigmoid colon. c Coronary section

Fig. 4

Intestinal ultrasonography image at the time of exacerbation. a The jejunum in the long axis image and the short axis image were indicated by the yellow and green arrowheads, respectively. Intestinal wall thickening was noted. b Color Doppler showed that blood flow was increasing on the serosal side of the jejunum (velocity range: 5 cm/second). c The sigmoid colon in the long axis image is indicated by the blue arrow. Intestinal wall thickening was observed. d Color Doppler showed no blood flow signals in the sigmoid colon (velocity range: 5 cm/second)

Fig. 5

Sigmoid colon on hospital day 8. Compared with Fig. 2, no significant change was observed

Fig. 6

Histologic analysis of biopsy specimens obtained from the ulcer of the sigmoid colonic mucosa on hospital day 8. A diffuse inflammatory cell infiltrate with neutrophils, lymphocytes, and plasma cells is observed, with no presence of granulomas (Hematoxylin & eosin, X200)

Fig. 7

Clinical course

Fig. 8

Sigmoid colon 10 months after colchicine initiation. The ulcer has disappeared, and the colonoscope can be passed through