Febrile Attacks With a Refractory Colonic Lesion

Abstract

Question: In April 2015, a 68-year-old woman with refractory inflammatory bowel disease was referred to our hospital for further examination. She had a history of pulmonary tuberculosis. Her symptoms of self-remitting fever, diarrhea, and abdominal pain initially occurred in 2005. Colonoscopy revealed multiple erosions with loss of mucosal vascularity throughout the colorectum. Although she was administered 4 g of mesalazine per day, her symptoms recurred. In 2014, ileocolonoscopy showed multiple ulcers and erosions in the terminal ileum and the ascending colon. She was administered 40 mg of oral prednisolone per day. Although her symptoms subsided, ileocolonic involvement remained active under ileocolonoscopy. In February 2015, she had a febrile attack with body temperature over 38 C and abdominal pain that spontaneously subsided within 2 days. In March, her symptoms repeated and laboratory data showed severe inflammation (white blood cell [WBC] count, 24,350/mL; C-reactive protein [CRP], 15.2 mg/dL) that lead to her referral to our hospital.On admission, her symptoms subsided and laboratory data only showed mild inflammatory reaction (WBC, 10,140/mL; CRP, 0.18 mg/dL). Immunologic tests revealed negative antinuclear antibody and positive QuantiFERON test. Ileocolonoscopy disclosed erythematous mucosa with multiple ulcers and erosions in the ascending colon ( Figure A) and in the terminal ileum ( Figure B). Other small intestinal involvement was not found under capsule endoscopy or balloon-assisted endoscopy.What is the diagnosis? Look on page 20 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and images in GI.

Gastrointestinal involvement in a patient with familial Mediterranean fever mimicking Crohn’s disease: a case report

“…The Tel-Hashomer criteria published in 1997 are often used for the diagnosis of FMF [15]. FMF involvement in gastrointestinal lesions has been considered rare, but recent reports have shown gastrointestinal involvement of FMF in the colon as well as in the small intestine, including the duodenum [4,5,7]. However, the endoscopic features of FMF with enterocolitis have not yet been clarified.…”

“…In small intestine lesions, several endoscopic findings such as redness, aphtha, erosion, and ulcer have been reported [7,11]. Furthermore, there are some reports of FMF patients with terminal ileum lesions mimicking Crohn's disease [3,4,8]. However, there have been no reports on the association of the site of genetic mutation with the morphology of gastrointestinal lesions.…”

“…In 2012, we first reported an IBDU patient with a heterozygous G304R mutation in the MEFV gene, who drastically responded to colchicine treatment alone [1]. Following this report, there have been several reports regarding IBDU patients with MEFV mutations responding to colchicine [2][3][4][5][6]. Based on these clinical cases, we advocated the existence of "MEFV gene-related enterocolitis" within the classification of IBDU.…”

Contradictory Effects of NLRP3 Inflammasome Regulatory Mechanisms in Colitis

“…FMF is mainly characterized by serositis and is rarely accompanied by intestinal mucosal lesions. However, in recent years, the number of cases with intestinal mucosal lesions has been increasing, and FMF-related enterocolitis should be distinguished from ulcerative colitis (UC) or CD [58][59][60][61][62][63].…”

Case report: Successful treatment with canakinumab of a patient with active ileitis associated with familial Mediterranean fever

“…Several reports of patients with FMF with gastrointestinal lesions have been published. 3,4 Furthermore, patients with enteritis of unknown cause have also been reported to have MEFV gene variants. These reports have highlighted the association between enteritis and FMF.…”