Ailesel Akdeniz Ateşi

Esra Nagehan Akyol Onder; Pelin Ertan

doi:10.34087/cbusbed.696594

Ailesel Akdeniz Ateşi

Abstract

Ailesel Akdeniz Ateşi (AAA) tekrarlayan ateş ve ateşe eşlik eden seröz zarların inflamasyonu ile karakterli otozomal resesif geçiş gösteren en sık otoinflamatuar hastalıktır. Özellikle Yahudiler, Araplar, Türkler ve Ermeniler’de sık olarak görülür. Kendi kendini sınırlayan ateş, karın ağrısı, göğüs ağrısı, eklem ağrısı ve şişliği, erizipel benzeri eritem atakları en sık belirti ve bulgulardır. Henoch Schönlein Purpura ve Poliarteritis Nodosa ile AAA birlikteliği sıktır. Tanı koymak amacı ile kullanılan spesifik bir test olmayıp hastalığın tanısı klinik ile konulur. Atak sırasında bakılan akut faz reaktanlarında artış ve atak dışı dönemde bunların normale dönmesi tanıyı destekler. AAA ayırıcı tanısında, diğer periyodik ateş sendromları ve diğer romatolojik hastalıklar, enfeksiyonlar, akut batın sendromları, maligniteler, porfiria gibi diğer hastalıklar göz önünde bulundurulmalıdır. 1972 yılından beri hastalığın tedavisinde etkin ilaç olarak kolşisin kullanılmaktadır. Kronik inflamasyona ikincil olarak gelişen AA (sekonder) amiloidoz, AAA’nın en ciddi komplikasyonudur. Kolşisin tedavisi atakların süre, sıklık ve şiddetini azalttığı gibi amiloidoz riskini de önler. Son yıllarda kolşisin tedavisini tolere edemeyen veya ilaca yanıt alınamayan hastalarda interlökin-1 antagonistlerinin kullanımı ile başarılı sonuçlar elde edilmiştir.

Keywords

Ailesel Akdeniz Ateşi,amiloidoz,kolşisin

References

Referans1. Ben-Chetrit, E, Levy, M. Familial Mediterranean fever. Lancet, 1998;351:659-64.
Referans2. Janeway, T.C, Mosenthal, H, Unusual paroxysmal syndrome, probably allied to recurrent vomiting, with a study of nitrogen metabolism. Trans Assoc Am Physicians, 1908; 23: 504518.
Referans3. Siegal, S, Benign paroxysmal peritonitis. Ann Intern Med, 1945; 23: 1-21.
Referans4. Marmaralı, A, Garip bir karın ağrısı sendromu. Türk Tıp Cem Mec, No:12, 1946.
Referans5. Mamou, H, Cattan, R, La maladie periodique. Sem Hop Paris 1952;1062-70.
Referans6. Heller, H, Sohar, E, Sherf, L, Familial Mediterranean fever. Arch Intern Med, 1958;102(1):50-71.
Referans7. Özkan, E, Okur, Ö, Ekmekçi, A, Özcan, R, Tag, T, A new approach to the treatment of periodic fever. Med Bull Istanbul, 1972; 5: 44-49.
Referans8. Goldfinger, S.E, Colchicine for Familial Mediterranean Fever. N Engl J Med, 1972; 287: 1302.

Referans9. Fonnesu, C, Cerquaglia, C, Giovinale, M, et al. Familial Mediterranean Fever: A review for clinical management. Joint Bone Spine, 2009; 76: 227-233.
Referans10. Onen, F, Familial Mediterranean fever. Rheumatol Int, 2006; 26(6): 489–96.
Referans11. Ben-Chetrit, E, Touitou, I, Familial Mediterranean Fever in the World. Arthritis&Rheumatism, 2009;61:1447-53.
Referans12. Lidar, M, Livneh, A, Familial Mediterranean Fever: clinical, molecular and advancements. The Journal of Medicine, 2007; 65 (9): 318-324.
Referans13. Turkısh FMF Study Group. Familial Mediterranean Fever in Turkey. Results of a Nationwide Multicenter Study. Medicine, 2005; 84: 1-11.
Referans14. Baskın, E, Saatçi, Ü, Familial Mediterranean Fever. Current Rheumatology Reviews, 2006; 2:101-108.
Referans15. Kasapçopur, Ö, Arısoy, N, Ailesel Akdeniz Ateşi ve diğer otoenflamatuar hastalıklar. Türk Pediatri Arşivi, 2006; 41; 9-17.
Referans16. The international FMF Consortium. Ancient missense mutations in a new member of the RoRet gene family are likely to cause FMF. Cell, 1997; 90:797-807.
Referans17. French FMF Consortium. A candidate gene for familial mediterranean fever. Nature Genetics, 1997; 17: 25-31.
Referans18. Soylemezoglu, O, Kandur, Y, Gonen, S, Düzova, A, Ozcakar, Z.B, Fidan, K, et al. Familial Mediterrenean fever gene mutation frequencies in a sample Turkish population. Clin Exp Rheumatol, 2016:102(6):97-100.
Referans19. Touitou I. Inheritance of autoinflammatory diseases: shifting pradigms and nomenclature. J Med Genet, 2013;50(6):349-59.
Referans20. Ozturk, C, Halıcıoglu, O, Coker, I, Gulez, N, Sutçuoglu, S, Karaca, N, et al. Association of clinical and genetical features in FMF with focus on MEFV strip assay sensitivity in 452 children from western Anatolia, Turkey. Clin Rheumatol, 2012;31:493-501.
Referans21. Arıcı, Z.S, Bilginer, Y, Özen, S, Ailesel Akdeniz Ateşi: Epidemiyoloji ve Genetik, Türkiye Klinikleri J Rheumatol-Special Topics, 2017;10(1):1-7.
Referans22. Manukyan, G, Rustam, A, Update on Pyrifunctions and mechanisms of Familial Mediterrenean Fever. Front. Microbiol, 2016;7:456.
Referans23. Wang, D.Q.H, Bonfrate, L, de Bari, O, Wang, T.Y, Portincasa, P, Familial Mediterranean fever: From pathogenesis to treatment. J Genet Syndr Gene Ther, 2014;5:248.
Referans24. Tamir, N, Langevitz, P, Zemer, D, Pras, E, Shinar, Y, Padeh, S, et al. Late-onset familial Mediterranean fever (FMF): a subset with distinct clinical, demographic, and molecular genetic characteristics. Am J Med Genet 1999;87(1):30-5.
Referans25. Özdel, S, Özçakar, Z.B, Şahin-Kunt, S, Elhan, A.E,Yalçınkaya, F. Late-onset disease is associated With a mild phenotype in children with familial Mediterranean fever. Clin Rheumatol, 2016 Jul;35(7):1837-40.
Referans26. Aydın, F, Özçakar, Z.B, Yalçınkaya, F, Çocuklarda Ailesel Akdeniz Ateşi Tanı ve Tedavisi, Türkiye Klinikleri J Rheumatol-Special Topics, 2017;10(1):46-54.
Referans27. Saatçi, Ü, Özen, S, Özdemir, S, Bakkaloğlu, A, Beşbaş, N, Topaloğlu, R, Arslan, Ş, Familial mediterranean fever in children: report of a large series and discussion of the risk and prognostic factors of amyloidosis. Eur J Pediatr, 1997; 156: 619-23.
Referans28. Sarı, I, Birlik, M, Kasifoğlu, T, Familial Mediterraneanfever: An updated review. Eur J Rheumatol, 2014;1(1):21-33.
Referans29. Tunca, M, Akar, S, Onen, F, Ozdogan, H, Kasapcopur, O, Yalcinkaya, F, Et al; Turkish FMF Study Group. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore), 2005; 84(1):1-11.
Referans30. Mor, A, Gal, R, Livneh, A, Abdominal and digestive system associations of familial mediterranean fever. Am J Gastroenterol, 2003; 98:2594-604.
Referans31. Lidar, M, Pras, M, Langevitz, P, Livneh A, Thoracic and Lung Involvement in Familial Mediaterranean Fever. Clin Chest Med, 2002; 23: 505-511.
Referans32. Langevitz, P, Zemer, D, Livneh, A, Shemer, J, Pras, M. Protracted febrile myalgia in patients with Familial Mediterranean Fever. J Rheumatol, 1994; 21: 1708-09.
Referans33. Yalcinkaya, F, Tekin, M, Çakar, N, Akar, E, Akar, N, Tümer,N, Familial Mediterrenean fever and systemic amyloidosis in untreated Turkish patients. Q J Med, 2000;93:681-4.
Referans34. Yalçinkaya, F, Tümer, N, Glomerular lesions other than amyloidosis in patients with familial Mediterranean fever. Nephrol Dial Transplant, 1999;14(1):21-3.
Referans35. Padeh, S, Periodic fever syndromes. Pediatr Clin N Am, 2005; 52:577-609.
Referans36. Sohar, E, Gafni,, J, Pras M, Heller, H, FMF: A survey of 470 cases and review of the literature. Am J Med, 1967;43:227-5.
Referans37. Livneh, A, Langevitz, P, Zemer, D et al. Criteria for the diagnosis of Familial mediterranean Fever. Arthritis-Rheumatism 1997; 40: 1879-85.
Referans38. Yalçınkaya, F, Ozen, S, Ozcakar, Z.B¸ Aktay, N, Çakar, N, Düzova, A, et al. A new set of criteria for the diagnosis of familial Mediterranean fever in childhood. Rheumatology, 2009;48: 395-98.
Referans39. Kondi, A, Hentgen, V, Piram, M, Letierce, A, Guillaume-Czitrom, S, Koné-Paut, Validation of the new paediatric criteria for the diagnosis of familial Mediterranean fever: data from a mixed population of 100 children from the French reference centre for auto-inflammatory disorders. Rheumatology (Oxford), 2010;49:2200-03.
Referans40. Slobodnick, A, Shah, B, Pillinger, M.H, Krasnokutsky, S, Colchicine: Old and New. The American Journal of Medicine, 2015;128:46170.
Referans41. Van der Hilst, J.C.H, Moutschen, M, Messiaen, P1, Lauwerys, B.R, Vanderschueren, S, Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature. Biologics, 2016;4(10):75-80.
Referans42. Mitroulis, I, Papadopoulos, VP, Konstantinidis, T, Ritis, K, Anakinra suppresses familial Mediterranean fever crises in a colchicine-resistant patient. The Journal of Medicine, 2008; 66: 489-91.

Details

Primary Language

Turkish

Subjects

Paediatrics, Rheumatology and Arthritis

Journal Section

Review

Authors

Esra Nagehan Akyol Onder ^*
0000-0003-0321-2204
Türkiye

Pelin Ertan
0000-0002-1882-5962
Türkiye

Publication Date

September 30, 2020

Submission Date

February 29, 2020

Acceptance Date

April 8, 2020

Published in Issue

Year 2020 Volume: 7 Number: 3

DOI

https://doi.org/10.34087/cbusbed.696594

IZ

https://izlik.org/JA25NL75YY

Cite

RIS / Bibtex

APA

Akyol Onder, E. N., & Ertan, P. (2020). Ailesel Akdeniz Ateşi. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal, 7(3), 413-417. https://doi.org/10.34087/cbusbed.696594

AMA

1.Akyol Onder EN, Ertan P. Ailesel Akdeniz Ateşi. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal. 2020;7(3):413-417. doi:10.34087/cbusbed.696594

Chicago

Akyol Onder, Esra Nagehan, and Pelin Ertan. 2020. “Ailesel Akdeniz Ateşi”. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal 7 (3): 413-17. https://doi.org/10.34087/cbusbed.696594.

EndNote

Akyol Onder EN, Ertan P (September 1, 2020) Ailesel Akdeniz Ateşi. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal 7 3 413–417.

IEEE

[1]E. N. Akyol Onder and P. Ertan, “Ailesel Akdeniz Ateşi”, CBU-SBED: Celal Bayar University-Health Sciences Institute Journal, vol. 7, no. 3, pp. 413–417, Sept. 2020, doi: 10.34087/cbusbed.696594.

ISNAD

Akyol Onder, Esra Nagehan - Ertan, Pelin. “Ailesel Akdeniz Ateşi”. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal 7/3 (September 1, 2020): 413-417. https://doi.org/10.34087/cbusbed.696594.

JAMA

1.Akyol Onder EN, Ertan P. Ailesel Akdeniz Ateşi. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal. 2020;7:413–417.

MLA

Akyol Onder, Esra Nagehan, and Pelin Ertan. “Ailesel Akdeniz Ateşi”. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal, vol. 7, no. 3, Sept. 2020, pp. 413-7, doi:10.34087/cbusbed.696594.

Vancouver

1.Esra Nagehan Akyol Onder, Pelin Ertan. Ailesel Akdeniz Ateşi. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal. 2020 Sep. 1;7(3):413-7. doi:10.34087/cbusbed.696594

Cited By

The effect of education given to Turkish adolescents with Familial Mediterranean fever on anxiety, depression, and quality of life

European Journal of Pediatrics

https://doi.org/10.1007/s00431-023-04975-5

Ailesel Akdeniz Ateşi Hastalarının MEFV Gen Mutasyon Tiplerinin Sıklığı ve Hastalarda Gen Mutasyonu ile Klinik Bulgular Arasındaki ilişkilerin Değerlendirilmesi

Dicle Tıp Dergisi

https://doi.org/10.5798/dicletip.1412077

Assessment of Health Literacy in Parents of Children With Familial Mediterranean Fever Using the Turkish Health Literacy Scale

Cureus

https://doi.org/10.7759/cureus.75693