Familial Mediterranean Fever and Accompanying Inflammatory Diseases: Effects On The Disease Severity Score

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.

Keywords

• Familial Mediterranean Fever
• Inflammatory Diseases
• Disease Severity Score
• Biological Agent

Ailevi Akdeniz Ateşine Eşlik Eden İnflamatuar Hastalıklar Ve Hastalık Ağırlık Skoruna Etkisinin Değerlendirilmesi

Abstract

Giriş ve Amaç: Ailevi Akdeniz Ateşi (AAA), çocukluk döneminde en sık görülen otoinflamatuar hastalıktır. Bazı inflamatuar hastalıkların AAA’ya eşlik edebildiği bilinmektedir. Çalışmamızda AAA tanılı hastalarda eşlik eden inflamatuar hastalıkları ve hastalık seyrine etkilerini inceledik. Gereç ve Yöntem: Çalışmamızda, 1 Ocak 2015-31 Aralık 2020 tarihleri arasında Yalçınkaya tanı kriterlerine göre AAA tanısı ile takip edilen 349 hastanın tıbbi kayıtları geriye dönük incelendi. AAA ile birliktelik gösteren inflamatuar hastalıkların Pras hastalık ağırlık skoruna etkisi araştırıldı. Bulgular: Çalışmaya dâhil edilen hastaların %45.8’inde hafif, %43.2’sinde orta ve %10.8’inde ağır hastalık mevcuttu. Çalışmaya dâhil edilen hastaların %14.8’inde eşlik eden inflamatuar bir hastalık tespit edildi (%5.7 IgA vasküliti, %3.7 sakroileit, %2.0 uzamış febril miyalji, %1.7 akut romatizmal ateş, %0.9). AAA’ya eşlik eden birden fazla inflamatuar hastalığın olduğu durumlar mevcuttu. Hastalık ağırlık skorunun, eşlik eden inflamatuar hastalığı olanlarda olmayanlara göre yüksek olduğu bulundu. Ek olarak eşlik eden inflamatuar hastalığı olan hastalarda ağır hastalık görülme oranının (%17.3) arttığı görüldü. Sonuç: Çalışmamızda eşlik eden inflamatuar hastalıkların AAA hastalığı ağırlık skorunu yükselttiği, ağırlık şiddetini artırdığı, bu hastalarda ataksız dönemdeki eritrosit sedimentasyon hızı değerlerinin yüksek seyrettiği ve biyolojik ajan kullanımının arttığı gösterilmiştir.

Keywords

• Ailevi Akdeniz Ateşi
• İnflamatuar Hastalık
• Hastalık Ağırlık Skoru
• Biyolojik Ajan

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