Background/Purpose: Familial Mediterranean Fever (FMF) is the most common auto-inflammatory disease with recurrent fever and serositis episodes. Abnormal pyrin protein due to MEFV gene mutations leads to clinical symptoms in FMF. Abdominal attacks happen due to the serosal inflammation in FMF. The sterile exudate which includes fibrin and polimorphonuclear cells occurs in the peritoneal space. Other gastrointestinal symptoms such as irritable bowel disease, colchicine related diarrhea and amyloidosis related malabsorption can be also detected. The rate of inflammatory bowel disease (IBD) is also shown to be increased in FMF. In recent years, some cases with FMF were reported with gastrointestinal involvement without amyloidosis, vasculitis and IBH. It is not yet known, whether the gastrointestinal involvement is a part of the disease or not. The aim of this study is to investigate the frequency of intestinal inflammation by using a noninvasive method, fecal calprotectin measurement, in pediatric FMF patients.

Methods: Sixty-five FMF patients, 30 healthy controls and 11 control patients with ulcerative colitis were included in the study. A standard survey which including gastrointestinal and other clinical symptoms and medications was used. MEFV mutations, whole blood count and CRP levels were recorded. Fecal calprotectin was studied with the ELISA method from the feces samples of the all patients. The upper cutoff value was determined as 200 ug/g.

Results: The patients were similar in terms of age and gender among groups. None of the FMF patients had clinical signs of the IBD. Fecal calprotectin levels of the FMF patients were found significantly higher than the healthy controls (174.8 ± 150.8 vs 52.9 ± 36.5, p< 0.001). In contrast, fecal calprotectin levels of the ulcerative colitis patients were significantly higher than the FMF patients (523.5 ± 183 vs 174.8 ± 150.8, p< 0.001). Even though, there are no IBD signs, the fecal calprotectin levels were higher than the cutoff point in 19 of the FMF patients. There was a correlation between fecal calprotectin levels and neutrophil/lymphocyte ratio (r=0.324, p=0.009).

Conclusion: Our results supported the subclinical intestinal inflammation in pediatric FMF patients. Underlying chronic auto-inflammatory process or continuous colchicine usage could result this observation. Further studies are needed to clarify the reason of the intestinal inflammation in FMF patients.

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/evaluation-of-intestinal-inflammation-in-children-with-fmf/