Case Report
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Erişkin Still Hastalığı Teşhisiyle İzlenen Hastada Makrofaj Aktivasyon Sendromu Gelişimi

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

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. 

References

  • 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.

Occurence Macrofage Activation Syndrome At the patient following Adult Still Disease

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

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. 

References

  • 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.
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Details

Primary Language Turkish
Journal Section Case Report
Authors

İsmail Boyraz

Selma Yazıcı This is me

Bunyamin Koc This is me

Ahmet Yıldız This is me

Umut Güven This is me

Publication Date April 15, 2015
Submission Date June 25, 2014
Acceptance Date August 23, 2014
Published in Issue Year 2015 Volume: 12 Issue: 1

Cite

Vancouver Boyraz İ, Yazıcı S, Koc B, Yıldız A, Güven U. Erişkin Still Hastalığı Teşhisiyle İzlenen Hastada Makrofaj Aktivasyon Sendromu Gelişimi. Harran Üniversitesi Tıp Fakültesi Dergisi. 2015;12(1):115-8.

Harran Üniversitesi Tıp Fakültesi Dergisi  / Journal of Harran University Medical Faculty