Erişkin Still Hastalığı Teşhisiyle İzlenen Hastada Makrofaj Aktivasyon Sendromu Gelişimi

Öz

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. 

Anahtar Kelimeler

• Artrit,Jüvenil,Makrofaj aktivasyon Sendromu

Occurence Macrofage Activation Syndrome At the patient following Adult Still Disease

Öz

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. 

Anahtar Kelimeler

• Arthritis,Juvenil,Macrophage Activation Syndrome

Kaynakça

1. 1. Efthimiou P, Kadavath S, Mehta B. Life-threatening complications of adult-onset Still's disease. Clinical Rheumatology 2014;33(3):305-14. 2. Pouchot J, Sampalis JS, Beaudet F, Carette S, Decary F, Salusinsky-Sternbach M et al. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 1991;70(2):118-36. 3. Ohta A, Yamaguchi M, Kaneoka H, Nagayoshi T, Hiida M. Adult Still's disease: review of 228 cases from the literature. J Rheumatol 1987;14(6):1139-46. 4. Efthimiou P, Kontzias A, Ward CM, Ogden NS. Adultonset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy? Nat Clin Pract Rheumatol 2007;3(6):328-35. 5. Cagatay Y, Gul A, Cagatay A, Kamali S, Karadeniz A, Inanc M et al. Adult-onset Still's disease. Int J Clin Pract 2009;63(7):1050-5. 6. Najam F, Fuchs HA. Cardiac tamponade: a lifethreatening complication of Still's disease. J Clin Rheumatol 2001;7(2):97-101. 7. Biron C, Chambellan A, Agard C, Denis M, Germaud P, Hamidou MA. Acute respiratory failure revealing adult-onset Still's disease: diagnostic value of low glycosylated ferritin level. Clin Rheumatol 2006;25(5):766-8. 8. Bagnari V, Colina M, Ciancio G, Govoni M, Trotta F. Adult-onset Still's disease. Rheumatol Int 2010;30(7):855-62. 9. Kontzias A, Efthimiou P. Adult-onset Still's disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs 2008;68(3):319-37. 10. Reddy VV, Myles A, Cheekatla SS, Singh S, Aggarwal A. Soluble CD25 in serum: a potential marker for subclinical macrophage activation syndrome in patients with active systemic onset juvenile idiopathic arthritis. Int J Rheum Dis 2014;17(3):261-7. 11. Kelly A, Ramanan AV. Recognition and management of macrophage activation syndrome in juvenile arthritis. Curr Opin Rheumatol 2007;19(5):477-81. 12. Szyper-Kravitz M. The hemophagocytic syndrome/macrophage activation syndrome: a final common pathway of a cytokine storm. Isr Med Assoc J 2009;11(10):633-4. 13. Ravelli A, Magni-Manzoni S, Pistorio A, Besana C, Foti T, Ruperto N, Viola S, Martini A. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 2005;146(5):598-604. 14. Clovis Artur A. Silva, Carlos Henrique M. Silva, Tereza Cristina M. V. Robazzi,Ana Paola N. Lotito,Alfredo Mendroni Junior,Cristina M. A. Jacob,Maria Helena B. Kiss. Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. J Pediatr (Rio J).2004;80(6):517-22. 15. Nadia EA, Carvalho JF, Bonfa E, Lotito AP, Silva CA. Macrophage activation syndrome associated with etanercept in a child with systemic onset juvenile idiopathic arthritis. Isr Med Assoc J 2009;11:635-36. 16. Stephan JL, Kone-Paut I, Galanbrun C, Mouy R, Bader Meunier B, Prieur AM. Reactive haemophagocytic syndrome in children with inflammatory disorders: a retrospective study 24 patients. Rheumatology(Oxford) 2001:40;1285-92. 17. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arc Dis Child 2001;85:421-26.