Investigation of cardiac autonomic dysfunction due to autoinflammation in patients followed up with familial mediterranean fever

Investigation of Cardiac Autonomic Dysfunction Due to Autoinflammation in Patients Followed Up with Familial Mediterranean Fever Baskent University Faculty of Medicine, Department of Child Health and Diseases, Thesis, Ankara, 2018 Objective: Familial Mediterranean Fever (FMF) is a systemic autoinflammatory disease characterized by recurrent fever, rash, abdominal pain, chest pain, and arthritis, resulting from a mutation in the MEFV gene located on chromosome 16p13.3, which encodes the pyrin protein. Many organs can be affected in the long-term course of the disease. Subclinical, chronic inflammation can also affect the autonomic nervous system. As a result, life-threatening ventricular arrhythmias and sudden death may occur as a result of myocardial involvement, arrhythmias and atherosclerotic changes over time. In recent studies, ECG parameters showing the presence of cardiac autonomic dysfunction in FMF patients have been investigated. For this purpose, it has been suggested that cardiac dysfunction parameters such as QT dispersion (QTcd) and JT dispersion (JTcd) on ECG may be useful. In our study, we aimed to investigate the importance of cardiac autonomic dysfunction by examining the epidemiological, clinical and laboratory characteristics of patients with FMF, as well as some ECG parameters which are accepted as electrocardiographic markers for ventricular arrhythmias. Material and method: As a group of 63 pediatric patients, aged 0-18 years, who applied to the Department of Pediatric Rheumatology, Baskent University Ankara Hospital, between January 2010 and December 2021, were diagnosed with Familial Mediterranean Fever (FMF), and were followed up, and at similar ages, for general control purposes. 63 healthy children without additional chronic disease who applied to the Department of Pediatric Cardiology were included in the study as the control group. FMF patients were selected from genetically homozygous or compound heterozygous patients who were followed for at least two years, did not have amyloidosis, and were stable in the attack-free period. The 12-lead ECGs of both the patient and the control group were taken and heart rate, QTcmin, QTcmax, JTcmin, JTcmax, QTc dispersion, and JTc dispersion were evaluated as ECG parameters. The relationship of ECG parameters with clinical, laboratory findings and genetic results was investigated. Results: The mean age of the patients was 12.1±4.24 years and the mean age of the control group was 13.21±2.90 years. The mean age at diagnosis of the patients was 5.76±4.43 years, the mean follow-up period was 6.41±4.06 years and the mean duration of colchicine treatment was 6.54±3.82 years. The gender, age, body weight and height levels of the patient and control groups were found to be similar (p>0.05). CRP (mg/L) and sedimentation measurement levels in the patient group were significantly higher than in the control group (p<0.05). In addition, heart rate (/min) and corrected JT dispersion (ms) measurement levels were found to be significantly higher in the patient group than in the controls (p<0.05). When the patients with at least one M694V mutation in at least one allele and the control group were compared with the ECG parameters, heart rate and JTcd measurement levels were found to be significantly higher (p<0.05). Conclusion: Our study showed that JTcd, which is accepted as an ECG indicator of the deterioration in the cardiac conduction system, is higher in FMF patients compared to the control group. This result suggests that although FMF patients do not show symptoms in childhood, the cardiac conduction system is affected. Routine cardiological evaluations of patients will be useful in the follow-up and treatment of FMF patients. Key words: Familial Mediterranean Fever, cardiac autonomic dysfunction Autoinflammation, QT dispersion, JT dispersion,