Factors triggering familial mediterranean fever attacks, do they really exist?

Several possible factors are hypothesized to trigger familial Mediterranean fever (FMF) attacks; however, there is no consensus on this matter. We aimed to identify these triggering factors and analyze their relationship with the Mediterranean fever gene mutation status. We prepared a questionnaire that included the most commonly mentioned possible trigger factors of familial Mediterranean fever. We administered a questionnaire to 882 patients. We used a questionnaire assessing the following: psychological stress, consumption of tea and coffee, relationship with menses, menopause and post-menopausal alleviation, seasonal changes, traveling for long durations, relocation, starvation, sleeplessness, cold exposure, fatigue, wind exposure, and humidity. The most frequent triggering factor for familial Mediterranean fever attacks was psychological stress (75.2%). Cold exposure was a statistically significant trigger in patients with exon 10 mutations. Humidity was a statistically significant trigger in patients with exon 2 mutations. Seasonal changes, traveling for long durations, relocation, and cold exposure were statistically significant triggers of familial Mediterranean fever attacks in patients with homozygous M694V mutations. Identifying trigger factors can lead to better preventive measures and personalized therapies to decrease familial Mediterranean fever attacks. Patients can significantly decrease the number of familial Mediterranean fever attacks they experience by managing psychological stress and avoiding physical factors such as cold exposure and fatigue. Determining the relationship between trigger factors and patients' Mediterranean fever gene mutation status can lead to personalized therapy for the prevention of familial Mediterranean fever attacks.

Anakinra in idiopathic recurrent pericarditis: a comprehensive case series and literature review.

OBJECTIVE: Idiopathic recurrent pericarditis (IRP) is defined by recurring episodes of pericardial inflammation without a known cause. This study investigates the safety and efficacy of anakinra, an interleukin­1 inhibitor, as a successful therapy for IRP in cases resistant to conventional treatment. METHODS: A retrospective evaluation of patients treated at our autoinflammatory center between 2011 and 2023 was conducted. Patient files were examined for demographic, clinical, and treatment response data, including nonsteroid anti-inflammatory drugs (NSAIDs), corticosteroids, and colchicine. Monogenic autoinflammatory disease screening was performed for Mediterranean Fever (MEFV), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase (MVK), nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3), and nucleotide-binding oligomerization domain-containing protein 2 (NOD2). Patients who experienced multiple episodes of pericarditis were diagnosed with recurrent pericarditis. The study evaluated anakinra treatment in IRP patients unresponsive to conventional therapy. RESULTS: The study included 21 participants, 9 (42.9%) female and 12 (57.1%) male. The average age of the participants was 43.1 ± 16.5 years. The MEFV mutation analysis revealed that 2 (9.5%) had a mutation in exon 10 and 4 (19.0%) had one in exon 2. Out of the 16 cases, 15 successfully discontinued steroid treatment. Four patients (19.0%) experienced injection site reactions. C­reactive protein (CRP) levels were measured at an average of 196 ± 67.8 mg/l before and 2.6 ± 3.15 mg/l after anakinra treatment. CONCLUSION: In conclusion, the study adds to the growing evidence for the efficacy of interleukin-1 inhibitors, such as anakinra, as a promising treatment modality for IRP in cases resistant to conventional treatment.

Cancer incidence in Familial Mediterranean Fever: A retrospective analysis.

OBJECTIVES: Familial Mediterranean Fever (FMF) is the most common hereditary monogenic fever syndrome that is characterized by recurrent attacks of fever and polyserositis. Anti-inflammatory drugs, with colchicine being the first-line therapy, have been used in the management of FMF. This study aims to evaluate the risk of cancer in Turkish FMF patients. METHODS: We retrospectively screened the cancer-related outcomes of our study group which consisted of Turkish FMF patients registered at our division. Cancer estimates of the Turkish population were published by the Turkish Ministry of Health in the Turkey Cancer Statistics Report 2018. Standardized incidence rates (SIR) were calculated to compare the cancer incidence observed in our study group with the expected cancer incidence of the Turkish population. Subgroup analyses were conducted on the subgroups, based on gender and usage of biological agents. RESULTS: Our study included 1734 FMF patients, 1054 (60.8 %) of whom were females. The total follow-up was 68,784 person-years. Cancer was observed in 35 (2 %) of these patients. Turkish FMF patients had a significantly lower incidence of cancer, compared with the overall Turkish population [SIR 0.64 (95 % CI 0.46-0.89), p < 0.01]. No significant association was found between cancer and biological agent therapies in FMF patients. CONCLUSIONS: Findings from our study indicate that the risk of cancer was decreased by 36 % in Turkish patients with FMF, compared with the outcomes of the overall Turkish population. Life-long exposure to anti-inflammatory drugs, primarily colchicine, may be the underlying reason for this outcome. Further studies are needed for the confirmation and explanation of this association.