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Published in:

01-04-2006 | Review

Familial Mediterranean fever

Author: Fatos Onen

Published in: Rheumatology International | Issue 6/2006

Abstract

Familial Mediterranean fever (FMF) is the most frequent hereditary inflammatory disease characterized by self-limited recurrent attacks of fever and serositis. It is transmitted in an autosomal recessive pattern and affects certain ethnic groups mainly Jews, Turks, Arabs, and Armenians. FMF is caused by mutations in MEFV gene, which encodes pyrin. This protein is expressed mainly in myeloid/monocytic cells and modulates IL-1β processing, NF-κB activation, and apoptosis. A mutated pyrin probably results in uncontrolled inflammation. The most devastating complication of FMF is amyloidosis, leading to chronic renal failure. M694V homozygocity, male gender and the α/α genotype of serum amyloid A1 gene are the currently established risk factors for development of amyloidosis. Daily colchicine is the mainstay of the therapy for the disease, resulting in complete remission or marked reduction in the frequency and duration of attacks in most patients. It is also effective in preventing and arresting renal amyloidosis.

Sohar E, Gafni J, Pras M, Heller H (1967) Familial Mediterranean fever. A survey of 470 cases and review of the literature. Am J Med 43:227–253CrossRefPubMed

Ancient missense mutations in a new member of the RoRet gene family are likely to cause familial Mediterranean fever (1997) The international FMF consortium. Cell 90:797–807

A candidate gene for familial Mediterranean fever (1997) The French FMF consortium. Nat Genet 17:25–31

Yilmaz E, Ozen S, Balci B, Duzova A, Topaloglu R, Besbas N, Saatci U, Bakkaloglu A, Ozguc M (2001) Mutation frequency of familial Mediterranean fever and evidence for a high carrier rate in the Turkish population. Eur J Hum Genet 9:553–555CrossRefPubMed

Shohat M, Magal N, Shohat T, Chen X, Dagan T, Mimouni A, Danon Y, Lotan R, Ogur G, Sirin A, Schlezinger M, Halpern GJ, Schwabe A, Kastner D, Rotter JI, Fischel-Ghodsian N (1999) Phenotype–genotype correlation in familial Mediterranean fever: evidence for an association between Met694Val and amyloidosis. Eur J Hum Genet 7:287–292CrossRefPubMed

Livneh A, Langevitz P, Shinar Y, Zaks N, Kastner DL, Pras M, Pras E (1999) MEFV mutation analysis in patients suffering from amyloidosis of familial Mediterranean fever. Amyloid 6:1–6PubMed

Ben-Chetrit E, Urieli-Shoval S, Calko S, Abeliovich D, Matzner Y (2002) Molecular diagnosis of FMF: lessons from a study of 446 unrelated individuals. Clin Exp Rheumatol 20:S25–S29PubMed

Majeed HA, El-Shanti H, Al-Khateeb MS, Rabaiha ZA (2002) Genotype/phenotype correlations in Arab patients with familial Mediterranean fever. Semin Arthritis Rheum 31:371–376CrossRefPubMed

Brik R, Shinawi M, Kepten I, Berant M, Gershoni-Baruch R (1999) Familial Mediterranean fever: clinical and genetic characterization in a mixed pediatric population of Jewish and Arab patients. Pediatrics 103:e70CrossRefPubMed

10.

Cattan D, Dervichian M, Thomas M, Dode C, Touitou I (2001) MEFV mutations and phenotype-genotype correlations in North African Jews and Armenians with familial Mediterranean fever. Isr Med Assoc J 3:803–804PubMed

11.

Gershoni-Baruch R, Shinawi M, Leah K, Badarnah K, Brik R (2001) Familial Mediterranean fever: prevalence, penetrance and genetic drift. Eur J Hum Genet 9:634–637CrossRefPubMed

12.

Yalcinkaya F, Cakar N, Misirlioglu M, Tumer N, Akar N, Tekin M, Tastan H, Kocak H, Ozkaya N, Elhan AH (2000) Genotype–phenotype correlation in a large group of Turkish patients with familial mediterranean fever: evidence for mutation-independent amyloidosis. Rheumatology (Oxford) 39:67–72CrossRef

13.

Akar N, Misiroglu M, Yalcinkaya F, Akar E, Cakar N, Tumer N, Akcakus M, Tastan H, Matzner Y (2000) MEFV mutations in Turkish patients suffering from familial Mediterranean fever. Hum Mutat 15:118–119CrossRefPubMed

14.

Touitou I (2001) The spectrum of familial Mediterranean fever (FMF) mutations. Eur J Hum Genet 9:473–483CrossRefPubMed

15.

Tunca M, Akar S, Onen F, Ozdogan H, Kasapcopur O, Yalcinkaya F, Tutar E, Ozen S, Topaloglu R, Yilmaz E, Arici M, Bakkaloglu A, Besbas N, Akpolat T, Dinc A, Erken E (2005) Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore) 84:1–11CrossRef

16.

Mansfield E, Chae JJ, Komarow HD, Brotz TM, Frucht DM, Aksentijevich I, Kastner DL (2001) The familial Mediterranean fever protein, pyrin, associates with microtubules and colocalizes with actin filaments. Blood 98:851–859CrossRefPubMed

17.

Masumoto J, Taniguchi S, Ayukawa K, Sarvotham H, Kishino T, Niikawa N, Hidaka E, Katsuyama T, Higuchi T, Sagara J (1999) ASC, a novel 22-kDa protein, aggregates during apoptosis of human promyelocytic leukemia HL-60 cells. J Biol Chem 274:33835–33838CrossRefPubMed

18.

Srinivasula SM, Poyet JL, Razmara M, Datta P, Zhang Z, Alnemri ES (2002) The PYRIN-CARD protein ASC is an activating adaptor for caspase-1. J Biol Chem 277:21119–21122CrossRefPubMed

19.

Martinon F, Burns K, Tschopp J (2002) The inflammasome: a molecular platform triggering activation of inflammatory caspases and processing of proIL-beta. Mol Cell 10:417–426CrossRefPubMed

20.

Chae JJ, Komarow HD, Cheng J, Wood G, Raben N, Liu PP, Kastner DL (2003) Targeted disruption of pyrin, the FMF protein, causes heightened sensitivity to endotoxin and a defect in macrophage apoptosis. Mol Cell 11:591–604CrossRefPubMed

21.

Masumoto J, Dowds TA, Schaner P, Chen FF, Ogura Y, Li M, Zhu L, Katsuyama T, Sagara J, Taniguchi S, Gumucio DL, Nunez G, Inohara N (2003) ASC is an activating adaptor for NF-kappa B and caspase-8-dependent apoptosis. Biochem Biophys Res Commun 303:69–73CrossRefPubMed

22.

Gumucio DL, Diaz A, Schaner P, Richards N, Babcock C, Schaller M, Cesena T (2002) Fire and ICE: the role of pyrin domain-containing proteins in inflammation and apoptosis. Clin Exp Rheumatol 20:S45–S53PubMed

23.

Ozen S, Uckan D, Baskin E, Besbas N, Okur H, Saatci U, Bakkaloglu A (2001) Increased neutrophil apoptosis during attacks of familial Mediterranean fever. Clin Exp Rheumatol 19:S68–S71PubMed

24.

Shoham NG, Centola M, Mansfield E, Hull KM, Wood G, Wise CA, Kastner DL (2003) Pyrin binds the PSTPIP1/CD2BP1 protein, defining familial Mediterranean fever and PAPA syndrome as disorders in the same pathway. Proc Natl Acad Sci USA 100:13501–13506CrossRefPubMed

25.

Wise CA, Gillum JD, Seidman CE, Lindor NM, Veile R, Bashiardes S, Lovett M (2002) Mutations in CD2BP1 disrupt binding to PTP PEST and are responsible for PAPA syndrome, an autoinflammatory disorder. Hum Mol Genet 11:961–969CrossRefPubMed

26.

Daniels M, Shohat T, Brenner-Ullman A, Shohat M (1995) Familial Mediterranean fever: high gene frequency among the non-Ashkenazic and Ashkenazic Jewish populations in Israel. Am J Med Genet 55:311–314CrossRefPubMed

27.

Ozen S, Karaaslan Y, Ozdemir O, Saatci U, Bakkaloglu A, Koroglu E, Tezcan S (1998) Prevalence of juvenile chronic arthritis and familial Mediterranean fever in Turkey: a field study. J Rheumatol 25:2445–2449PubMed

28.

Onen F, Sumer H, Turkay S, Akyurek O, Tunca M, Ozdogan H (2004) Increased frequency of familial Mediterranean fever in Central Anatolia, Turkey. Clin Exp Rheumatol 22:S31–S33PubMed

29.

Rogers DB, Shohat M, Petersen GM, Bickal J, Congleton J, Schwabe AD, Rotter JI (1989) Familial Mediterranean fever in Armenians: autosomal recessive inheritance with high gene frequency. Am J Med Genet 34:168–172CrossRefPubMed

30.

Livneh A, Langevitz P, Zemer D, Padeh S, Migdal A, Sohar E, Pras M (1996) The changing face of familial Mediterranean fever. Semin Arthritis Rheum 26:612–627CrossRefPubMed

31.

Schwabe AD, Peters RS (1974) Familial Mediterranean fever in Armenians. Analysis of 100 cases. Medicine (Baltimore) 53:453–462CrossRef

32.

Heller H, Gafni J, Michaeli D, Shahin N, Sohar E, Ehrlich G, Karten I, Sokoloff L (1966) The arthritis of familial Mediterranean fever (FMF). Arthritis Rheum 9:1–17PubMedCrossRef

33.

Majeed HA, Rawashdeh M (1997) The clinical patterns of arthritis in children with familial Mediterranean fever. QJM 90:37–43PubMed

34.

Sneh E, Pras M, Michaeli D, Shanin N, Gafni J (1997) Protracted arthritis in familial Mediterranean fever. Rheumatol Rehabil X 16:102–106CrossRef

35.

Salai M, Langevitz P, Blankstein A, Zemmer D, Chechick A, Pras M, Horoszowski H (1993) Total hip replacement in familial Mediterranean fever. Bull Hosp Jt Dis 53:25–28PubMed

36.

Besbas N, Ozdemir S, Saatci I, Bakkaloglu A, Ozen S, Saatci U (1999) Sacroiliitis in familial Mediterranean fever: an unusual presentation in childhood. Turk J Pediatr 41:387–390PubMed

37.

Langevitz P, Zemer D, Livneh A, Shemer J, Pras M (1994) Protracted febrile myalgia in patients with familial Mediterranean fever. J Rheumatol 21:1708–1709PubMed

38.

Pras M, Bronshpigel N, Zemer D, Gafni J (1982) Variable incidence of amyloidosis in familial Mediterranean fever among different ethnic groups. Johns Hopkins Med J 150:22–26PubMed

39.

Konstantopoulos K, Kanta A, Tzoulianos M, Dimou S, Sotsiou F, Politou M, Loukopoulos D (2001) Familial Mediterranean fever phenotype II in Greece. Isr Med Assoc J 3:862–863PubMed

40.

Kutlay S, Yilmaz E, Koytak ES, Tulunay OO, Keven K, Ozcan M, Erturk S (2001) A case of familial Mediterranean fever with amyloidosis as the first manifestation. Am J Kidney Dis 38:E34PubMed

41.

Melikoglu M, Ozdogan H, Korkmaz C, Kasapcopur O, Arisoy N, Akkus S, Fresko Z, Yazici H (2000) A survey of phenotype II in familial Mediterranean fever. Ann Rheum Dis 59:910–913CrossRefPubMed

42.

Cazeneuve C, Ajrapetyan H, Papin S, Roudot-Thoraval F, Genevieve D, Mndjoyan E, Papazian M, Sarkisian A, Babloyan A, Boissier B, Duquesnoy P, Kouyoumdjian JC, Girodon-Boulandet E, Grateau G, Sarkisian T, Amselem S (2000) Identification of MEFV-independent modifying genetic factors for familial Mediterranean fever. Am J Hum Genet 67:1136–1143PubMed

43.

Booth DR, Gillmore JD, Booth SE, Pepys MB, Hawkins PN (1998) Pyrin/marenostrin mutations in familial Mediterranean fever. QJM 91:603–606CrossRefPubMed

44.

Samuels J, Aksentijevich I, Torosyan Y, Centola M, Deng Z, Sood R, Kastner DL (1998) 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) 77:268–297CrossRef

45.

Yalcinkaya F, Akar N, Misirlioglu M (1998) Familial Mediterranean fever—amyloidosis and the Val726Ala mutation. N Engl J Med 338:993–994CrossRefPubMed

46.

Tekin M, Yalcinkaya F, Cakar N, Akar N, Misirlioglu M, Tastan H, Tumer N (2000) MEFV mutations in multiplex families with familial Mediterranean fever: is a particular genotype necessary for amyloidosis? Clin Genet 57:430–434CrossRefPubMed

47.

Yilmaz E, Balci B, Kutlay S, Ozen S, Erturk S, Oner A, Besbas N, Bakkaloglu A (2003) Analysis of the modifying effects of SAA1, SAA2 and TNF-alpha gene polymorphisms on development of amyloidosis in FMF patients. Turk J Pediatr 45:198–202PubMed

48.

Bakkaloglu A, Duzova A, Ozen S, Balci B, Besbas N, Topaloglu R, Ozaltin F, Yilmaz E (2004) Influence of serum amyloid A (SAA1) and SAA2 gene polymorphisms on renal amyloidosis, and on SAA/C-reactive protein values in patients with familial mediterranean fever in the Turkish population. J Rheumatol 31:1139–1142PubMed

49.

Touitou I, Picot MC, Domingo C, Notarnicola C, Cattan D, Demaille J, Kone-Paut I (2001) The MICA region determines the first modifier locus in familial Mediterranean fever. Arthritis Rheum 44:163–169CrossRefPubMed

50.

Flatau E, Kohn D, Schiller D, Lurie M, Levy E (1982) Schonlein-Henoch syndrome in patients with familial Mediterranean fever. Arthritis Rheum 25:42–47PubMedCrossRef

51.

Glikson M, Galun E, Schlesinger M, Cohen D, Haskell L, Rubinow A, Eliakim M (1989) Polyarteritis nodosa and familial Mediterranean fever: a report of 2 cases and review of the literature. J Rheumatol 16:536–539PubMed

52.

Ozen S, Ben-Chetrit E, Bakkaloglu A, Gur H, Tinaztepe K, Calguneri M, Turgan C, Turkmen A, Akpolat I, Danaci M, Besbas N, Akpolat T (2001) Polyarteritis nodosa in patients with familial Mediterranean fever (FMF): a concomitant disease or a feature of FMF? Semin Arthritis Rheum 30:281–287CrossRefPubMed

53.

Akar S, Goktay Y, Akinci B, Tekis D, Biberoglu K, Birlik M, Onen F, Tunca M, Akkoc N (2005) A case of familial Mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations. Rheumatol Int 25:60–64CrossRefPubMed

54.

Schwartz T, Langevitz P, Zemer D, Gazit E, Pras M, Livneh A (2000) Behcet’s disease in familial Mediterranean fever: characterization of the association between the two diseases. Semin Arthritis Rheum 29:286–295CrossRefPubMed

55.

Touitou I, Magne X, Molinari N, Navarro A, Quellec AL, Picco P, Seri M, Ozen S, Bakkaloglu A, Karaduman A, Garnier JM, Demaille J, Kone-Paut I (2000) MEFV mutations in Behcet’s disease. Hum Mutat 16:271–272CrossRefPubMed

56.

Atagunduz P, Ergun T, Direskeneli H (2003) MEFV mutations are increased in Behcet’s disease (BD) and are associated with vascular involvement. Clin Exp Rheumatol 21:S35–S37PubMed

57.

Gershoni-Baruch R, Broza Y, Brik R (2003) Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch-Schonlein purpura. J Pediatr 143:658–661CrossRefPubMed

58.

Ozen S, Bakkaloglu A, Yilmaz E, Duzova A, Balci B, Topaloglu R, Besbas N (2003) Mutations in the gene for familial Mediterranean fever: do they predispose to inflammation? J Rheumatol 30:2014–2018PubMed

59.

Ehrenfeld M, Brzezinski A, Levy M, Eliakim M (1987) Fertility and obstetric history in patients with familial Mediterranean fever on long-term colchicine therapy. Br J Obstet Gynaecol 94:1186–1191PubMed

60.

Ehrenfeld EN, Polishuk WZ (1970) Gynecological aspects of recurrent polyserositis (familial Mediterranean fever, periodic disease). Isr J Med Sci 6:9–13PubMed

61.

Ben-Chetrit E, Levy M (2003) Reproductive system in familial Mediterranean fever: an overview. Ann Rheum Dis 62:916–919CrossRefPubMed

62.

Gang N, Drenth JP, Langevitz P, Zemer D, Brezniak N, Pras M, van der Meer JW, Livneh A (1999) Activation of the cytokine network in familial Mediterranean fever. J Rheumatol 26:890–897PubMed

63.

Frensdorff A, Sohar E, Heller H (1961) Plasma fibrinogen in familial Mediterranean fever. Ann Intern Med 55:448–455PubMed

64.

Moutereau S, Narwa R, Matheron C, Vongmany N, Simon E, Goossens M (2004) An improved electronic microarray-based diagnostic assay for identification of MEFV mutations. Hum Mutat 23:621–628CrossRefPubMed

65.

Delague V, Kriegshauser G, Oberkanins C, Megarbane A (2004) Reverse hybridization vs. DNA sequencing in the molecular diagnosis of Familial Mediterranean fever. Genet Test 8:65–68CrossRefPubMed

66.

Drenth JP, van der Meer JW (2001) Hereditary periodic fever. N Engl J Med 345:1748–1757CrossRefPubMed

67.

Hoffman HM, Mueller JL, Broide DH, Wanderer AA, Kolodner RD (2001) Mutation of a new gene encoding a putative pyrin-like protein causes familial cold autoinflammatory syndrome and Muckle–Wells syndrome. Nat Genet 29:301–305CrossRefPubMed

68.

McDermott MF, Aksentijevich I, Galon J, McDermott EM, Ogunkolade BW, Centola M, Mansfield E, Gadina M, Karenko L, Pettersson T, McCarthy J, Frucht DM, Aringer M, Torosyan Y, Teppo AM, Wilson M, Karaarslan HM, Wan Y, Todd I, Wood G, Schlimgen R, Kumarajeewa TR, Cooper SM, Vella JP, Amos CI, Mulley J, Quane KA, Molloy MG, Ranki A, Powell RJ, Hitman GA, O’Shea JJ, Kastner DL (1999) Germline mutations in the extracellular domains of the 55 kDa TNF receptor, TNFR1, define a family of dominantly inherited autoinflammatory syndromes. Cell 97:133–144CrossRefPubMed

69.

Aksentijevich I, Galon J, Soares M, Mansfield E, Hull K, Oh HH, Goldbach-Mansky R, Dean J, Athreya B, Reginato AJ, Henrickson M, Pons-Estel B, O’Shea JJ, Kastner DL (2001) The tumor-necrosis-factor receptor-associated periodic syndrome: new mutations in TNFRSF1A, ancestral origins, genotype–phenotype studies, and evidence for further genetic heterogeneity of periodic fevers. Am J Hum Genet 69:301–314CrossRefPubMed

70.

Dode C, Andre M, Bienvenu T, Hausfater P, Pecheux C, Bienvenu J, Lecron JC, Reinert P, Cattan D, Piette JC, Szajnert MF, Delpech M, Grateau G (2002) The enlarging clinical, genetic, and population spectrum of tumor necrosis factor receptor-associated periodic syndrome. Arthritis Rheum 46:2181–2188CrossRefPubMed

71.

Aganna E, Hammond L, Hawkins PN, Aldea A, McKee SA, van Amstel HK, Mischung C, Kusuhara K, Saulsbury FT, Lachmann HJ, Bybee A, McDermott EM, La Regina M, Arostegui JI, Campistol JM, Worthington S, High KP, Molloy MG, Baker N, Bidwell JL, Castaner JL, Whiteford ML, Janssens-Korpola PL, Manna R, Powell RJ, Woo P, Solis P, Minden K, Frenkel J, Yague J, Mirakian RM, Hitman GA, McDermott MF (2003) Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypes. Arthritis Rheum 48:2632–2644CrossRefPubMed

72.

Hull KM, Drewe E, Aksentijevich I, Singh HK, Wong K, McDermott EM, Dean J, Powell RJ, Kastner DL (2002) The TNF receptor-associated periodic syndrome (TRAPS): emerging concepts of an autoinflammatory disorder. Medicine (Baltimore) 81:349–368CrossRef

73.

Drenth JP, Haagsma CJ, van der Meer JW (1994) Hyperimmunoglobulinemia D and periodic fever syndrome. The clinical spectrum in a series of 50 patients. International Hyper-IgD Study Group. Medicine (Baltimore) 73:133–144

74.

Drenth JP, Cuisset L, Grateau G, Vasseur C, van de Velde-Visser SD, de Jong JG, Beckmann JS, van der Meer JW, Delpech M (1999) Mutations in the gene encoding mevalonate kinase cause hyper-IgD and periodic fever syndrome. International Hyper-IgD Study Group. Nat Genet 22:178–181CrossRefPubMed

75.

Muckle TJ, Wellsm (1962) Urticaria, deafness, and amyloidosis: a new heredo-familial syndrome. Q J Med 31:235–248

76.

Prieur AM, Griscelli C, Lampert F, Truckenbrodt H, Guggenheim MA, Lovell DJ, Pelkonnen P, Chevrant-Breton J, Ansell BM (1987) A chronic, infantile, neurological, cutaneous and articular (CINCA) syndrome. A specific entity analysed in 30 patients. Scand J Rheumatol Suppl 66:57–68PubMedCrossRef

77.

Padeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, Migdal A, Pras M, Passwell JH (1999) Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: clinical characteristics and outcome. J Pediatr 135:98–101CrossRefPubMed

78.

Goldfinger SE (1972) Colchicine for familial Mediterranean fever. N Engl J Med 287:1302PubMed

79.

Zemer D, Revach M, Pras M, Modan B, Schor S, Sohar E, Gafni J (1974) A controlled trial of colchicine in preventing attacks of familial Mediterranean fever. N Engl J Med 291:932–934PubMedCrossRef

80.

Livneh A, Zemer D, Langevitz P, Laor A, Sohar E, Pras M (1994) Colchicine treatment of AA amyloidosis of familial Mediterranean fever. An analysis of factors affecting outcome. Arthritis Rheum 37:1804–1811PubMedCrossRef

81.

Zemer D, Pras M, Sohar E, Modan M, Cabili S, Gafni J (1986) Colchicine in the prevention and treatment of the amyloidosis of familial Mediterranean fever. N Engl J Med 314:1001–1005PubMedCrossRef

82.

Lidar M, Scherrmann JM, Shinar Y, Chetrit A, Niel E, Gershoni-Baruch R, Langevitz P, Livneh A (2004) Colchicine nonresponsiveness in familial Mediterranean fever: clinical, genetic, pharmacokinetic, and socioeconomic characterization. Semin Arthritis Rheum 33:273–282CrossRefPubMed

83.

Lidar M, Kedem R, Langevitz P, Pras M, Livneh A (2003) Intravenous colchicine for treatment of patients with familial Mediterranean fever unresponsive to oral colchicine. J Rheumatol 30:2620–2623PubMed

84.

Ben-Chetrit E, Levy M (1998) Colchicine: 1998 update. Semin Arthritis Rheum 28:48–59CrossRefPubMed

85.

Amital H, Ben-Chetrit E (2004) Therapeutic approaches to familial Mediterranean fever. What do we know and where are we going to? Clin Exp Rheumatol 22:S4–S7PubMed

86.

Ozkaya N, Yalcinkaya F (2003) Colchicine treatment in children with familial Mediterranean fever. Clin Rheumatol 22:314–317CrossRefPubMed

87.

Tunca M, Tankurt E, Akbaylar Akpinar H, Akar S, Hizli N, Gonen O (1997) The efficacy of interferon alpha on colchicine-resistant familial Mediterranean fever attacks: a pilot study. Br J Rheumatol 36:1005–1008CrossRefPubMed

88.

Tunca M, Akar S, Soyturk M, Kirkali G, Resmi H, Akhunlar H, Gonen O, Gallimore JR, Hawkins PN, Tankurt E (2004) The effect of interferon alpha administration on acute attacks of familial Mediterranean fever: a double-blind, placebo-controlled trial. Clin Exp Rheumatol 22:S37–S40PubMed

89.

Calguneri M, Apras S, Ozbalkan Z, Ozturk MA (2004) The efficacy of interferon-alpha in a patient with resistant familial Mediterranean fever complicated by polyarteritis nodosa. Intern Med 43:612–614CrossRefPubMed

90.

Seyahi E, Ozdogan H, Masatlioglu S, Yazici H (2002) Successful treatment of familial Mediterranean fever attacks with thalidomide in a colchicine resistant patient. Clin Exp Rheumatol 20:S43–S44PubMed

91.

Drenth JP, Vonk AG, Simon A, Powell R, van der Meer JW (2001) Limited efficacy of thalidomide in the treatment of febrile attacks of the hyper-IgD and periodic fever syndrome: a randomized, double-blind, placebo-controlled trial. J Pharmacol Exp Ther 298:1221–1226PubMed

92.

Sampaio EP, Sarno EN, Galilly R, Cohn ZA, Kaplan G (1991) Thalidomide selectively inhibits tumor necrosis factor alpha production by stimulated human monocytes. J Exp Med 173:699–703PubMedCrossRef

93.

Milledge J, Shaw PJ, Mansour A, Williamson S, Bennetts B, Roscioli T, Curtin J, Christodoulou J (2002) Allogeneic bone marrow transplantation: cure for familial Mediterranean fever. Blood 100:774–777CrossRefPubMed

94.

Touitou I, Ben-Chetrit E, Gershoni-Baruch R, Grateau G, Kastner DL, Kone-Paut I, Livneh A, Manna R, Mansour I, Ozdogan H, Ozen S, Sarkisian T, Tunca M, Yalcinkaya F (2003) Allogenic bone marrow transplantation: not a treatment yet for familial Mediterranean fever. Blood 102:409CrossRefPubMed

95.

Keven K, Sengul S, Kutlay S, Ekmekci Y, Anadol E, Nergizoglu G, Ates K, Erturk S, Erbay B (2004) Long-term outcome of renal transplantation in patients with familial Mediterranean fever amyloidosis: a single-center experience. Transplant Proc 36:2632–2634CrossRefPubMed

96.

Altiparmak MR, Pamuk ON, Ataman R, Serdengecti K (2004) Continuous ambulatory peritoneal dialysis in familial Mediterranean fever amyloidosis patients with end-stage renal failure: a single-centre experience from Turkey. Nephron Clin Pract 98:c119–c123CrossRefPubMed

97.

Rawashdeh MO, Majeed HA (1996) Familial Mediterranean fever in Arab children: the high prevalence and gene frequency. Eur J Pediatr 155:540–544PubMedCrossRef

Title: Familial Mediterranean fever
Author: Fatos Onen
Publication date: 01-04-2006
Publisher: Springer-Verlag
Published in: Rheumatology International / Issue 6/2006
Print ISSN: 0172-8172
Electronic ISSN: 1437-160X
DOI: https://doi.org/10.1007/s00296-005-0074-3

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