Association of familial Mediterranean fever in Turkish children with inflammatory bowel disease

Abstract

Aim: Familial Mediterranean fever (FMF) and inflammatory bowel disease (IBD) carry similar clinical and biological properties. Both are characterized with chronic inflammation attacks and neutrophil migration and impaired apoptosis mechanism are present in the areas of damage in both conditions. In our study, we aimed to determine the frequency of association of FMF in patients with IBD, to compare the demographic, clinical, laboratory and treatment response properties in these patients with the ones in other IBD patients and to determine association of FMF especially in treatment-resistant patients. Material and Methods:Fifty-three patients who were being followed up with a diagnosis of IBD aged between 0 and 18 years were included in the study. The patient group included the patients who were diagnosed with IBD according to clinical, serological, endoscopic and histopathological criteria, who were being followed up and whose therapies were continuing. Genetic analysis in terms of MEFV gene mutations was performed in all patients with a diagnosis of IBD. Acute phase reactants, complete blood count, immunoglobulin levels, stool analysis, "perinuclear anti-neutrophil cytoplasmic antibodies" (pANCA) and "anti-Saccharomyces cerevisiae antibodies" (ASCA) were studied at the time of diagnosis. The diagnosis of FMF was made according to detailed history, physical examination findings, laboratory tests and the results of genetic analyses in terms of MEFV gene mutations in accordance with the criteria defined in 2009. Results:We found that FMF accompanied in 14 (26.4%) of the patients who had a diagnosis of IBD. 3 of these 14 patients in whom FMF accompanied were being followed up with a diagnosis of Crohn disease and 11 were being followed up with a diagnosis of ulcerative colitis. All of these patients had MEFV gene mutation. These mutations included M694V (50%), K695R (21.4%), M680I (14.3%) and R202Q (14.3%) in order of frequency. When the laboratory data were compared between the patients who had a diagnosis of IBD alone and who had a diagnosis of IBD plus FMF, it was observed that the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) values were statistically significantly higher in the IBD+FMF group.Conclusions: FMF is a common condition in the Turkish population and M694V mutation is found most commonly. In our study, this status did not change in cases where FMF accompanied IBD, but K695R mutation was found more frequently compared to FMF alone. We think that it should be kept in mind that other inflammatory conditions including mainly FMF may accompany IBD, if a case of IBD does not have an expected course or is resistant to treatment. (Türk Ped Arş 2014; 49: 198-202)

Familial Mediterranean fever patients with hidradenitis suppurativa

“…MEFV mutations have also been reported in other autoimmune diseases. There are several reports indicating MEFV mutations are increased among IBD patients . Recently, E148Q heterozygous MEFV gene mutation in two cases of PG with aseptic arthritis is reported …”

The Influence of Concomitant Disorders on Disease Severity of Familial Mediterranean Fever in Children

“…Several studies showed associations between FMF and IBD; however, to our knowledge, relevance on FMF severity was not studied before. 12,13 In a study, coexistence of FMF with Chron disease (CD) was correlated with younger diagnosis age of IBD; however, intestinal involvement and complication rates were not affected by presence of FMF. On the other hand, FMF attack frequency and persistent proteinuria were higher in FMF patients with CD than only FMF patients; this may be due to additive inflammation of CD on FMF course.…”

Fecal calprotectin in children with familial Mediterranean fever in the attack‐free period

“…Beşer et al . showed that 15.4% of patients with FMF had clinical and histopathological findings consistent with IBD . Fidder et al .…”