Sistemik başlangıçlı juvenil idiyopatik artrit tanılı olguların değerlendirilmesi

Öz

GİRİŞ ve AMAÇ: Sistemik başlangıçlı juvenile idiyopatik artrit (JİA) nadir görülen bir JİA alt tipi olarak kabul edilir ve diğer JİA alt gruplarından farklı olarak otoinflamatuar hastalık grubunda değerlendirilir. Ülkemizde sistemik başlangıçlı JİA ile ilgili az sayıda çalışma bulunmaktadır. Bu çalışmada kliniğimizde sistemik başlangıçlı JİA tanılı olguların klinik ve laboratuvar özelliklerinin araştırılması amaçlanmıştır.
YÖNTEM ve GEREÇLER: Kliniğimizde takip edilen 303 JİA tanılı olgu tarandı ve 18 sistemik başlangıçlı JİA tanılı olgu (%5,9) tespit edildi. Olguların verileri elektronik dosya kayıtlarından geriye dönük olarak taranarak elde edildi.
BULGULAR: Olguların yaşları ortanca 11,7 yıl (5,3-20 yıl) ve kız erkek oranı 4/14 idi. Tanı yaşları ortanca 4,9 yıl (1,5-15,2 yıl) ve tanıda gecikme süresi ortanca 4 hafta (2 hafta-10,8 yıl) olarak saptandı. En sık başvuru yakınması olguların tümünde görülen ateş ve artrit (n=18, %100) idi. Bir olguda monoartrit görülürken diğer olgularda birden fazla eklem tutulumu vardı. En sık görülen form monosiklik formdu (n=7, %38,8). Olgularda görülen en sık komplikasyon boy kısalığı (n=4, %22,2) idi ve makrofaj aktivasyon sendromu 2 olguda (%11,1) gelişti. Tedavide en sık steroidler (n=17, %94,4) kullanılırken biyolojik tedavi 7 olguda (%38,8) kullanıldı. Bu olguların son tedavilerine bakıldığında; 5 olgunun tocilizumab, bir olgunun canakinumab aldığı, kalan 1 olgunun ise takibi bıraktığı saptandı. Takipten çıkan bir olgu dışında tüm olgularda remisyon sağlandı.
TARTIŞMA ve SONUÇ: Sistemik başlangıçlı JİA, diğer JİA alt gruplarına kıyasla daha az olarak görülmektedir. Hastalık enfeksiyon hastalıkları ve diğer inflamatuar hastalıklarla karışabildiği için tanı gecikebilmektedir. Kortikosteroid tedavisine çoğunlukla olumlu yanıt alınmakla birlikte steroide dirençli olgularda anti IL-6 ve anti IL-1 ilaçlar etkili olabilmektedir

Anahtar Kelimeler

• Sistemik başlangıçlı juvenil idiopatik artrit,otoinflamatuar hastalık

Evaluation of patients with systemic onset juvenile idiopathic arthritis

Öz

INTRODUCTION: Systemic juvenile idiopathic arthritis (sJIA) is a rare, systemic inflammatory disease classified as a subtype of JIA. There are few studies on sJIA in our country. The aim of this study was to investigate the clinical and laboratory features of patients with sJIA.
METHODS: Three hundred three cases with JIA followed up in our clinic were screened, 18 of them (5.9%) diagnosed with sJIA wereincluded to our study. The data was obtained from the electronic file records retrospectively.
RESULTS: The median age of the patients was 11.7 years (5.3-20 years), the female/male ratio was 4/14, and the median diagnosis age was 4.9 years (1.5-15.2 years). The median diagnosis delay was 4 weeks (2 weeks-10.8 years). The most common complaints were fever and arthritis which were seen in all cases (n = 18, 100%). One patient had monoarthritis while the other cases had more than one joint involvement. The most common form was the monocyclic form (n = 7, 38.8%). The most common complications were short stature and macrophage activation syndrome which developed in 2 cases. Almost all patients were treated by corticosteroids (n = 17, 94.4%), biological therapy was used in 7 patients (38.8%). Biological treatments were tocilizumab in five patients, canakinumab in one and anakinra in 1 one patient. Remission was achieved in all cases except one patient who was out of follow-up.
DISCUSSION AND CONCLUSION: SJIA can be challenging to diagnose. Fever and rash in sJIA clinic may inadvertently be attributed to infectious disease. Corticosteroids were the main treatment modalities in treatment, whereas in resistant cases biologicals were highly effective and safe.

Anahtar Kelimeler

• Systemic onset juvenile idiopathic arthritis,autoinflammatory disease

Kaynakça

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