Demographic, clinical and laboratory characteristics of patients with familial mediterranean fever with chest pain attack

Aim: Comparison of demographic, clinical and laboratory sections of familial Mediterranean patients with chest pain attacks and without chest pain attacks. Materials and Methods: In our study, the electronic records and files of 548 patients who were diagnosed with familial Mediterranean fever (FMF) between October 2016 and July 2022 to Ankara University Faculty of Medicine, Department of Pediatrics Rheumatology were evaluated retrospectively. Results: The mean age was 72 (3-217) months, and the delay in diagnosis was 26 (0-191) months in 548 patients of whom 289 (52.7) were girls. The history of consanguineous marriage was 25%, and the family history of FMF was 66%. In the attack, 93.4% of the patients had fever, 89% had abdominal pain, 53% had joint pain, 25% had acute arthritis, and 24.8% had chest pain. The mean leukocyte count during the attack was 10503.93±4329.86/mm3, CRP 69.94±57.46 mg/L, sedimentation rate 32.33±21.91 mm/hour, and fibrinogen 4.23±1.16 g/L. The most common mutations detected in patients; M694V (72.7%), M680I (19%), V726A (17%), E148Q (14%) were mutations. The mean colchicine dose of the patients was 1.31±0.40 mg/kg/day, the annual attack frequency before colchicine was 12 (16.11±11.61) attacks, and the annual attack frequency after colchicine treatment was 1(1.78±3.15) attack. The number of attacks after colchicine was found to be significantly lower than before colchicine use (p<0.001). Chest pain was found in 136 of 548 patients diagnosed with familial Mediterranean fever, and 74 (54.4%) of the patients were girls. The median age at diagnosis of patients with chest pain was 86 (14-209) months. The age at diagnosis of these patients was found to be older than those without chest pain (p<0.01). Fever (91%) and abdominal pain (91.2%) were the most common findings accompanying chest pain. Prolonged febrile myalgia was found to be higher in these patients than in the patients without chest pain (p=0.007). There was no significant difference between the frequency of mutations in patients with and without chest pain. The annual number of attacks before colchicine, colchicine dose, and the rate of colchicine-resistant patients were found to be higher in patients with chest pain than those without chest pain (p<0.05). The mean Mor disease severity score of the patients with chest pain was 2.27±0.74, and the ISSF disease severity score was 1.94±1.08. These severity scores of patients with chest pain were higher than without chest pain (p<0.001). Conclusion: In conclusion, patients with chest pain in FMF showed more severe disease characteristics than patients without chest pain, with higher colchicine dose requirement, greater colchicine resistance, and higher disease severity scores.