Erişkin Still Hastalığı Teşhisiyle İzlenen Hastada Makrofaj Aktivasyon Sendromu Gelişimi

Year 2015, Volume: 12 Issue: 1, 115 - 118, 15.04.2015

İsmail Boyraz , Selma Yazıcı Bunyamin Koc Ahmet Yıldız Umut Güven

Abstract

Still hastalığı sistemik başlangıçlı juvenil idiopatik artritin (SOJİA) analoğu olan sistemik enflamatuar bir
hastalıktır. Makrofaj aktivasyon sendromu (MAS) SOJİA'da görülen mortalite ve morbiditenin önemli
nedenlerinden biridir. 2 yıldır Still hastalığı teşhisi ile izlenen 20 yaşındaki bayan hasta methotrexate 15
mg/hafta, Plaquenil 200 mg, folbiol tb almaktaydı. Hasta alt ekstremitede yaygın cilt kızarıkları ve artralji
şikayetleriyle polikliniğimize başvurdu. Tetkiklerinde akut faz reaktanlarında yükselme vardı ve hastada
Still aktivasyonu düşünülerek Salazopyrin tb eklendi. 1 hafta sonra 3-4 gündür devam eden boğaz ağrısı ve
yutma güçlüğü şikayetiyle tekrar kliniğimize başvurdu. Hastanın takibinde genel durumunda ve laboratuvar
değerlerinde bozulma görüldü. Hastanın sonraki muayenesinde yaygın lenfadenopati, splenomegali
saptandı, oral alımı kesilmişti. Bulantı, kusma ve ateşi devam ediyordu. Hastada Makrofaj Aktivasyon
Sendromu düşünülerek hematoloji konsültasyonu istendi ve yatışından 1 hafta sonra teşhis amaçlı yapılan
kemik iliği biyopsisi ile Hemofagositik sendrom teşhisi konuldu. Hastaya pulse steroid (1 gr/gün), İVİG ve
Siklosporin 100 mg 2*1 tedavisi düzenlendi. 

Keywords

Artrit, Jüvenil, Makrofaj aktivasyon Sendromu

Occurence Macrofage Activation Syndrome At the patient following Adult Still Disease

Abstract

Still Disease is systemic inflammatory disease as a kind of Systemic Juvenil Rheumatoid Arthritis.
Macrophage Activation Syndrome is a reason of mortality and morbidity at SOJIA. The 20 years old patient
followed with the diagnosis of Still Disease for two years had been taking Methotrexate 15 mg/week,
Plaquenil 200 mg/day. The patient turned to our clinic with complaints of common skin redness and
arthralgia. In her test results, acute phase reactants were too high and when it was thought as illness
activation, salazopyrine was added to the patient's treatment. The patient came to our clinic one week later
with the complaints of throat pain lasting for 3-4 days and difficulty in swallowing. At the follow of the
patient, deterioration of the patient's general condition and laboratory measures was found. We found during
patient's physical examination common LAP and splenomegaly. Oral intake had stopped. Nausea, vomiting
and temparature was going on. We wanted to consult to hematology department as to whether MAC
syndrome diagnose was correct. Within a week of admitting the patient, bone marrow biopsy was made to
correct MAC diagnosis and it proved to be true. The medical treatment was arranged as pulse steroid (1
gr/day), IVIG and Cyclosporin 100 mg 2*1. 

Keywords

Arthritis, Juvenil, Macrophage Activation Syndrome

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