Which physical findings are characteristic of familial Mediterranean fever (FMF)?

1. Padeh S, Berkun Y. Familial Mediterranean fever. Curr Opin Rheumatol. 2016 May 8. [Medline].

2. Manukyan G, Aminov R. Update on Pyrin Functions and Mechanisms of Familial Mediterranean Fever. Front Microbiol. 2016. 7:456. [Medline].

3. Sönmez HE, Batu ED, Özen S. Familial Mediterranean fever: current perspectives. J Inflamm Res. 2016. 9:13-20. [Medline].

4. Gohar F, Orak B, Kallinich T, Jeske M, Lieber M, von Bernuth H, et al. Secretory Activity of Neutrophils Correlates With Genotype in Familial Mediterranean Fever. Arthritis Rheumatol. 2016 Jun 22. [Medline].

5. Apostolidou E, Skendros P, Kambas K, Mitroulis I, Konstantinidis T, Chrysanthopoulou A, et al. Neutrophil extracellular traps regulate IL-1β-mediated inflammation in familial Mediterranean fever. Ann Rheum Dis. 2016 Jan. 75 (1):269-77. [Medline].

6. Özen S, Batu ED, Demir S. Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management. Front Immunol. 2017. 8:253. [Medline]. [Full Text].

7. Majeed HA, El-Khateeb M, El-Shanti H, et al. The spectrum of familial Mediterranean fever gene mutations in Arabs: report of a large series. Semin Arthritis Rheum. 2005 Jun. 34(6):813-8. [Medline].

8. Gershoni-Baruch R, Brik R, Shinawi M, Livneh A. The differential contribution of MEFV mutant alleles to the clinical profile of familial Mediterranean fever. Eur J Hum Genet. 2002 Feb. 10(2):145-9. [Medline]. [Full Text].

9. Tunca M, Akar S, Onen F, et al. Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine (Baltimore). 2005 Jan. 84(1):1-11. [Medline].

10. Tomiyama N, Higashiuesato Y, Oda T, et al. MEFV mutation analysis of familial Mediterranean fever in Japan. Clin Exp Rheumatol. 2008 Jan-Feb. 26(1):13-7. [Medline].

11. Kim S, Ikusaka M, Mikasa G, et al. Clinical study of 7 cases of familial Mediterranean fever with MEFV gene mutation. Intern Med. 2007. 46(5):221-5. [Medline]. [Full Text].

12. Lidar M, Livneh A. Familial Mediterranean fever: clinical, molecular and management advancements. Neth J Med. 2007 Oct. 65(9):318-24. [Medline]. [Full Text].

13. Hentgen V, Grateau G, Stankovic-Stojanovic K, Amselem S, Jéru I. Familial Mediterranean fever in heterozygotes: are we able to accurately diagnose the disease in very young children?. Arthritis Rheum. 2013 Jun. 65(6):1654-62. [Medline].

14. Lidar M, Kedem R, Mor A, et al. Arthritis as the sole episodic manifestation of familial Mediterranean fever. J Rheumatol. 2005 May. 32(5):859-62. [Medline].

15. Salehzadeh F, Azami A, Motezarre M, Nematdoust Haghi R, Ahmadabadi F. Neurological Manifestations in Familial Mediterranean Fever: a Genotype-Phenotype Correlation Study. Open Access Rheumatol. 2020. 12:15-19. [Medline]. [Full Text].

16. Mukhin NA, Kozlovskaya LV, Bogdanova MV, Rameev VV, Moiseev SV, Simonyan AK. Predictors of AA amyloidosis in familial Mediterranean fever. Rheumatol Int. 2015 Jan 14. [Medline].

17. [Guideline] Giancane G, Ter Haar NM, Wulffraat N, Vastert SJ, Barron K, Hentgen V, et al. Evidence-based recommendations for genetic diagnosis of familial Mediterranean fever. Ann Rheum Dis. 2015 Jan 27. [Medline].

18. Kuky O, Livneh A, Ben-David A, et al. Familial Mediterranean Fever (FMF) with proteinuria: Clinical Features, Histology, Predictors, and Prognosis in a cohort of 25 patients. J Rheumatol. 12/2013. 40:2083-7.

19. Polat A, et al; FMF Arthritis Vasculitis and Orphan Disease Research in Pediatric Rheumatology (FAVOR). Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever--a randomized controlled noninferiority trial. Arthritis Res Ther. 2016 Apr 7. 18:85. [Medline].

20. [Guideline] Ozen S, Demirkaya E, Erer B, Livneh A, Ben-Chetrit E, Giancane G, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016 Apr. 75 (4):644-51. [Medline]. [Full Text].

21. Lidar M, Kedem R, Langevitz P, et al. Intravenous colchicine for treatment of patients with familial Mediterranean fever unresponsive to oral colchicine. J Rheumatol. 2003 Dec. 30(12):2620-3. [Medline].

22. Sönmez HE, Batu ED, Bilginer Y, Özen S. Discontinuing colchicine in symptomatic carriers for MEFV (Mediterranean FeVer) variants. Clin Rheumatol. 2017 Feb. 36 (2):421-425. [Medline].

23. Makay B, Gulez N. Long-term follow-up of paediatric MEFV carriers. Clin Rheumatol. 2018 Feb 13. [Medline].

24. Mor A, Pillinger MH, Kishimoto M, et al. Familial Mediterranean fever successfully treated with etanercept. J Clin Rheumatol. 2007 Feb. 13(1):38-40. [Medline].

25. Roldan R, Ruiz AM, Miranda MD, et al. Anakinra: new therapeutic approach in children with Familial Mediterranean Fever resistant to colchicine. Joint Bone Spine. 2008 Jul. 75(4):504-5. [Medline].

26. Ben-Zvi I, Kukuy O, Giat E, Pras E, Feld O, Kivity S, et al. Anakinra for Colchicine-Resistant Familial Mediterranean Fever: A Randomized, Double-Blind, Placebo-Controlled Trial. Arthritis Rheumatol. 2017 Apr. 69 (4):854-862. [Medline].

27. Hashkes PJ, Spalding SJ and Giannin EH, et al. Rilonacept for Colchicine-Resistant or -Intolerant Familial Mediterranean Fever. A Randomized Trial. Ann Intern Med. 2012. 157:533-541. [Medline]. [Full Text].

28. Tunca M, Akar S, Soyturk 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 Jul-Aug. 22(4 Suppl 34):S37-40. [Medline].

29. Tweezer-Zaks N, Rabinovich E, Lidar M, et al. Interferon-alpha as a treatment modality for colchicine- resistant familial Mediterranean fever. J Rheumatol. 2008 Jul. 35(7):1362-5. [Medline].

30. Calguneri M, Apras S, Ozbalkan Z, et al. The efficacy of continuous interferon alpha administration as an adjunctive agent to colchicine-resistant familial Mediterranean fever patients. Clin Exp Rheumatol. 2004 Jul-Aug. 22(4 Suppl 34):S41-4. [Medline].

31. van der Hilst JCh, Moutschen M, Messiaen PE, Lauwerys BR, Vanderschueren S. Efficacy of anti-IL-1 treatment in familial Mediterranean fever: a systematic review of the literature. Biologics. 2016. 10:75-80. [Medline].

32. Ilaris (canakinumab) [package insert]. East Hanover, NJ: Novartis Pharmaceutical Co. September, 2016. Available at [Full Text].

33. Kacar M, Savic S, van der Hilst JCH. The Efficacy, Safety and Tolerability of Canakinumab in the Treatment of Familial Mediterranean Fever: A Systematic Review of the Literature. J Inflamm Res. 2020. 13:141-149. [Medline]. [Full Text].

34. [Guideline] Ward MM, Deodhar A, Akl EA, Lui A, Ermann J, et al. American College of Rheumatology/Spondylitis Association of America/Spondyloarthritis Research and Treatment Network 2015 Recommendations for the Treatment of Ankylosing Spondylitis and Nonradiographic Axial Spondyloarthritis. Arthritis Rheumatol. 2016 Feb. 68 (2):282-98. [Medline]. [Full Text].

35. Ben-Chetrit E, Berkun Y, Ben-Chetrit E, et al. The outcome of pregnancy in the wives of men with familial mediterranean fever treated with colchicine. Semin Arthritis Rheum. 2004 Oct. 34(2):549-52. [Medline].

John O Meyerhoff, MD Clinical Scholar in Rheumatology, Department of Medicine, Sinai Hospital of Baltimore

John O Meyerhoff, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Elliot Goldberg, MD Dean of the Western Pennsylvania Clinical Campus, Professor, Department of Medicine, Lewis Katz School of Medicine at Temple University

Elliot Goldberg, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Carlos J Lozada, MD Director of Rheumatology Fellowship Training Program, Professor of Clinical Medicine, Department of Medicine, Division of Rheumatology and Immunology, University of Miami, Leonard M Miller School of Medicine

Carlos J Lozada, MD is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Received honoraria from Pfizer for consulting; Received grant/research funds from AbbVie for other; Received honoraria from Heel for consulting.