Abstract:

Objective: Familial Mediterranean Fever (FMF) stands as the most prevalent autoinflammatory disorder in childhood. It is well-established that certain inflammatory conditions may coexist with FMF. Within the scope of our investigation, we examined the inflammatory diseases accompanying FMF in diagnosed pediatric patients, as well as their potential impact on the disease progression. Materials and Methods: We retrospectively reviewed the medical records of 349 patients diagnosed with FMF based on the Yalçınkaya diagnostic criteria, who were followed between January 1, 2015, and December 31, 2020. The potential impact of inflammatory diseases coexisting with FMF on the Pras disease severity score was investigated. Results: Among the patients included in the study, 45.8% exhibited mild disease, 43.2% had moderate disease, and 10.8% had severe disease. Among the study participants, 14.8% were found to have coexisting inflammatory diseases. Specifically, IgA vasculitis was present in 5.7% of cases, sacroiliitis in 3.7%, prolonged febrile myalgia in 2.0%, acute rheumatic fever in 1.7%. Some cases showed the coexistence of multiple inflammatory conditions alongside FMF. It was observed that the disease severity score was higher in patients with coexisting inflammatory diseases. Additionally, the rate of severe disease was found to be increased in patients with coexisting inflammatory diseases (17.3%). Conclusion: Our study demonstrated that coexisting inflammatory diseases elevate the disease severity score and increase the intensity of FMF. Furthermore, patients with coexisting inflammatory diseases showed higher erythrocyte sedimentation rate values during attack-free periods and an increased use of biological agents.

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