Fatma AYDIN, Tuba KURT, Nilüfer TEKGÖZ, Müge SEZER, Özge BAŞARAN, Nilgün ÇAKAR, Banu ACAR

Sistemik Juvenil İdiyopatik Artritte Son On Yılda Neler Değişti?

Amaç: Sistemik juvenil idiyopatik artrit (sJİA), JİA’nın yedi alt tipinden biridir. Bu çalışmanın amacı, kliniğimizdeki sJİA hastalarının özelliklerini, seyrini ve sonuçlarını araştırmak ve son on yılda sJİA’da nelerin değiştiğini belirlemektir. Gereç ve Yöntemler: Mart 2002 ile Nisan 2019 arasında takip edilen sJİA hastalarının dosyaları geriye dönük olarak değerlendirildi ve verileri kaydedildi. Bulgular: Çalışma süresince kliniğimizde 36 hastaya sJİA tanısı konuldu (19 erkek). Hastalığın başlangıcında hastaların ortalama yaşı 81.7±50.1 aydı. Başvuru esnasında en sık saptanan bulgular ateş (%100), artrit (%83.3), döküntü (% 66.7) ve hepatosplenomegali (%38.9)’du. Yedi hasta (%19.4) sJİA’ya bağlı gelişen makrofaj aktivasyon sendromu (MAS) tanısı aldı. Tüm hastalara kortikosteroid tedavisi verildi. 29 hastaya ek tedaviler (hastalığı modifiye edici ilaçlar, intravenöz immünoglobulin ve biyolojik ajanlar) verildi. Kliniğimizde 2011 yılından itibaren anti interlökin (İL) -1 ajanlar kullanılmaktadır. Bu grupta 26 (%72.2) hasta 2011 yılından sonra tanı almıştır. 2011 yılından sonra tanı alan ve biyolojik ajan tedavisi kullanılan hastalarda (n: 26) 2011 yılından önce tanı alan hastalara (n:10) göre steroid maruziyet süresi anlamlı olarak kısalmıştır (p = 0.001 ). Sonuç: Biyolojik ajanların sJİA tedavisinde kullanılmaya başlamasından sonra steroid maruziyet süresi önemli ölçüde azalmıştır. Anti İL-1 ve anti İL-6 tedavilerinin sJİA hastalarında öncelikli tedaviler arasında düşünülmesi hem hızlı hastalık kontrolünü sağlamak hem de ilaçlara bağlı ortaya çıkabilecek yan etkileri azaltmak açısından önemlidir.

Anahtar Kelimeler:

Biyolojik ajanlar, Klinik özellikler, Makrofaj aktivasyon sendromu, Sistemik juvenil idiyopatik artrit

What Has Changed Over the Last Decade in Systemic Juvenile İdiopathic Arthritis?

Objective: Systemic juvenile idiopathic arthritis (sJIA) is one of seven subtypes of JIA. The aim of this study was to investigate the characteristics, the course and the outcome of sJIA patients in our clinic and to identify what has changed over the last decade in sJIA. Material and Methods: Files of sJIA patients that were followed between March 2002 and April 2019 were evaluated. Results: During the study period 36 patients were diagnosed with sJIA in our clinic (19 were male). Mean age of patients was 81.7±50.1 months at the onset of disease. Most common presenting features were fever (100%), arthritis (83.3%), rash (66.7%) and hepatosplenomegaly (38.9%). Seven patients (19.4%) were diagnosed as macrophage activation syndrome (MAS) due to sJIA. All patients had received corticosteroids. Additional treatments (disease-modifying drugs, intravenous immunoglobulin and biological agents) were given to 29 patients. In our clinic, anti-IL-1 agents have been used since 2011. In this group 26 (72.2%) patients were diagnosed after 2011. After the usage of biological agents, the duration of steroid exposure significantly decreased (p= 0.001). Conclusion: After the introduction of biological agents in the treatment of sJIA, the duration of steroid exposure decreased significantly. Considering anti interleukin (IL)-1 and anti IL-6 treatments among the priority treatments in sJIA patients is important in terms of both providing rapid disease control and reducing side effects related to drugs.

Keywords:

Biological agents, Clinical features, Macrophage activation syndrome, Systemic juvenile idiopathic arthritis,

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