Birinci Basamakta Ailevi Akdeniz Ateşine Genel Yaklaşım

Ailevi Akdeniz Ateşi (AAA); otozomal resesif geçiş özelliği olan ve özellikle Sefardik Yahudiler, Türkler, Ermeniler ve Araplar gibi Doğu Akdeniz halklarında yaygın rastlanılan bir otoinflamatuar periyodik hastalıktır. AAA; tekrarlayan ateş ve özellikle periton, plevra ve eklemlerde, poliserözite sekonder olarak ortaya çıkan ağrı ataklarıyla karakterize bir hastalıktır. Bulgular ani başlangıçlıdır, sıklıkla 20 yaşından önce ortaya çıkar ve genellikle 1 ile 4 gün içinde kendiliğinden kaybolur. Tüm dünyadaki AAA’li hastaların büyük çoğunluğu ülkemizde görüldüğü için, birinci basamakta çalışan hekimlerin AAA’li hastalara yaklaşımı önemlidir

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Familial Mediterranean fever (FMF), an autoinflammatory periodic disorder, is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly Sephardic Jews, Turks, Armenians and Arabs. FMF is characterized by recurrent attacks of fever and pain secondary to polyserositis, especially peritoneum, pleura and joints. The symptoms are sudden onset, usually happen before the age of 20 and generally decrease spontaneously within 1-4 days. Since a large proportion of all the FMF patients in the world live in Turkey, approach to patients with FMF in the primary care is discussed in this article
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  • Bernot A, Clepet C, Dasilva C, on behalf of the French FMF consortium. A candidate gene for familial mediterranean fever. Nat Genet 1997; 17(1): 25-31.
  • Ben-Chetrit E, Touitou I. Familial Mediterranean Fever in the World. Arthritis Rheum 2009; 61(10): 1447– 53.
  • Tunca M, Akar S, Onen F, et al. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 2005; 84(1): 1–11.
  • Cobankara V, Fidan G, Turk T, Zencir M, Colakoglu M, Ozen S. The prevalence of familial Mediterranean fever in the Turkish province of Denizli: a field study with a zero patient design. Clin Exp Rheumatol 2004; 22(4 suppl 34): 527–30.
  • Onen F, Sumer H, Turkay S, Akyurek O, Tunca M, Ozdogan H. Increased frequency of familial Mediterranean fever in Central Anatolia, Turkey. Clin Exp Rheumatol. 2004; 22(4 Suppl 34): 31-4.
  • Kisacik B, Yildirim B, Tasliyurt T, et al. Increased frequency of familial Mediterranean fever in northern Turkey: a population-based study. Rheumatol Int. 2009; 29(11): 1307-17.
  • Etem E, Deveci SD, Erol D, Yuce H, Elyas H. Familial Mediterranean Fever: A Retrospective Clinical and Molecular Study in the East of Anatolia Region of Turkey. Open Rheumatol J 2010; 4: 1-6.
  • Yilmaz E, Ozen S, Balci B, et al. Mutation frequency of Familial Mediterranean Fever and evidence for a high carrier rate in the Turkish population. Eur J Hum Genet 2001; 9(7): 553-5.
  • Tüzün A, Dursun A, Ateş Y, et al. Ailesel Akdeniz Ateşi düşünülen 110 vakada sık görülen MEFV gen mutasyonları analiz sonuçları ve klinik bulgularla korelasyonu. Gülhane Tıp Dergisi 2004; 46 (3): 238-41.
  • Coker I, Colak A, Yolcu I, et al. MEFV gene mutation spectrum in familial Mediterranean fever (FMF): a single center study in the Aegean region of Turkey. Z Rheumatol 2011; 70(6): 511-6.
  • Örün E, Yalcınkaya F, Özkaya N, Akar N, Gökçe H. Ailevi Akdeniz Ateşi (AAA) Hastalığında akut faz yanıtı ile tümör nekrozis faktör-α, interlökin-8 ve interlökin-6 düzeylerinin değerlendirilmesi. Ankara Üniversitesi Tıp Fakültesi Mecmuası 2002; 55(2): 123-8.
  • Lin E, Calvono SE, Lowry SF. Inflammatory cytokines and cell response in surgery. Surgery 2000; 127(2):117-26.
  • Dinarello CA. IL-1: discoveries, controversies and future directions. Eur J Immunol 2010; 40(3): 599-606.
  • Dinarello CA. Interleukin-1 beta and the autoinflammatory diseases. N Engl J Med 2009; 360(23): 2467-70.
  • Bilgen SA, Kilic L, Akdogan A, et al. Effects of anti-tumor necrosis factor agents for Familial Mediterranean Fever patients with Chronic Arthritis and/or Sacroiliitis who were resistant to colchicine treatment. J Clin Rheumatol 2011; 17(7): 358-65.
  • Saatci U, Bakkaloglu A, Ozen S, Besbas N. Familial Mediterranean fever and amyloidosis in children. Acta Paediatr 1993; 82(8): 705-6.
  • Majeed HA, Rawashdeh M, El-Shanti H, Oubain H, Khuri-Bulos N, Shahin HM. Familial Mediterranean fever in children: the expanded clinical profile. QJM 1999; 92(6): 309-18.
  • Guza G, Kanbay M, Ozturk MA. Current perspectives on familial Mediterranean fever. Curr Opin Infect Dis 2009; 22(3): 309–15.
  • Gang N, Drenth JP, Langevitz P, et al. Activation of the cytokine network in familial Mediterranean Fever. J Rheumatol 1999; 26(4): 890–7.
  • Kivity S, Danilesko I, Ben-Zvi I, et al. Serum amyloid A levels in kidney-transplanted patients with familial Mediterranean fever-amyloidosis. Isr Med Assoc J 2011; 13(4): 202-5.
  • Samuels J, Aksentijevich I, Torosyan Y, et al. Familial Mediterranean fever at the millennium. Clinical spectrum, ancient mutations, and a survey of 100 American referrals to the National Institutes of Health. Medicine (Baltimore) 1998; 77(4): 268–30.
  • Ergüven M, Emeksiz C, Deveci M, Özlü SG. Relation between microalbuminuria and gene mutations in familial Mediterranean fever. Turk J Pediatr 2008; 50(4): 326-5.
  • Koşan C. Ailevi Akdeniz Ateşine tanısal yaklaşım. AÜTD 2003; 35: 1-6.
  • Livneh A, Langevitz P, Zemer D, et al. Criteria for the diagnosis of Familial Mediterranean Fever. Arthritis Rheum 1997; 40(10): 1879-85.
  • Guzel S, Andican G, Seven A, et al. Acute phase response and oxidative stress status in Familial Mediterranean Fever (FMF). Mod Rheumatol 2012; 22(3): 431-7.
  • Drenth JP, Haagsma CJ, van der Meer JW. Hyperimmunoglobulinemia D and periodic fever syndrome. The clinical spectrum in a series of 50 patients. International Hyper-IgD Study Group. Medicine (Baltimore) 1994; 73(3): 133-44.
  • McDermott EM, Smillie DM, Powell RJ. Clinical spectrum of familial Hibernian fever: A 14-year follow- up study of the index case and extended family. Mayo Clin Proc 1997; 72(9): 806-12.
  • Padeh S, Brezniak N, Zemer D, et al. Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: Clinical characteristics and outcome. J Pediatr 1999; 135(1): 98-4.
  • Atagündüz P, Ergun T, Direskeneli H. MEFV mutations are increased in Behcet's disease (BD) and are associated with vascular involvement. Clin Exp Rheumatol 2003; 21(4 Suppl 30):S35-7.
  • Tekin M, Yalcinkaya F, Tumer N, Akar N, Misirlioglu M, Cakar N. Clinical, laboratory and molecular characteristics of children with familial Mediterranean fever-associated vasculitis. Acta Paediatr 2000; 89(2): 177-6.
  • Zemer D, Livneh A, Danon YL, Pras M, Sohar E. Long-term colchicine treatment in children with familial Mediterranean fever. Arthritis Rheum 1991; 34(8): 973-7.
  • Lidar M, Kedem R, Langevitz P, Pras M, Livneh A. Intravenous colchicine for treatment of patients with familial Mediterranean fever unresponsive to oral colchicine. J Rheumatol. 2003; 30(12): 2620-3.
  • Zemer D, Pras M, Sohar E, Modan B, Cabili S, Gafni J. Colchicine in the prevention and treatment of amyloidosis of familial Mediterranean fever. N Engl J Med 1986; 314(16): 1001-5.
  • Zemer D, Revach M, Pras M, et al. A controlled trial of colchicine in preventing attacks of familial Mediterranean fever. N Engl J Med 1974; 291(18): 932-4.
  • Rabinovitch O, Zemer D, Kukia E, Sohar E, Mashiach S. Colchicine treatment at conception and pregnancy: two hundred thirty-one pregnancies in patients with Familial Mediterranean Fever. Am J Reprod Immunol 1992; 28(3-4): 245-6.
  • Ehrenfeld M, Brzezinski A, Levy M, Eliakim M. Fertility and obstetric history in patients with familial Mediterranean fever on long-term colchicine therapy. Br J Obstet Gynaecol 1987; 94(12): 1186-6.
  • Tunca M, Tankurt E, Akbaylar Akpinar H, Akar S, Hizli N, Gönen O. The efficacy of interferon alpha on colchicine-resistant familial Mediterranean fever attacks: a pilot study. Br J Rheumatol. 1997;36(9):1005-8.
  • Tunca M, Akar S, Soytürk M, et al. The effect of interferon alpha administration on acute attacks of familial Mediterranean fever: A double-blind, placebo-controlled trial. Clin Exp Rheumatol. 2004; 22(4 Suppl 34): 37-40.
  • Seyahi E, Ozdogan H, Celik S, et al. Treatment options in colchicine resistant familial Mediterranean fever patients: thalidomide and etanercept as adjunctive agents. Clin Exp Rheumatol 2006; 24 (5 suppl 42): 99-5.
  • Ozgocmen S, Ozçakar L, Ardicoglu O, et al. Familial Mediterranean fever responds well to infliximab: single case experience. Clin Rheumatol 2006; 25(1): 83-7.
  • Calligaris L, Marchetti F, Tommasini A, Ventura A. The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever. Eur J Pediatr 2008; 167(6): 695-6.