Clinical Rheumatology

, Volume 28, Issue 1, pp 41–46 | Cite as

The frequency of sacroiliitis in familial Mediterranean fever and the role of HLA-B27 and MEFV mutations in the development of sacroiliitis

  • Timuçin Kaşifoğlu
  • Cüneyt Çalışır
  • Döndü Ü. Cansu
  • Cengiz KorkmazEmail author
Original Article


The objective of this study was to investigate the frequency of sacroiliitis in familial Mediterranean fever (FMF) patients and the role of HLA-B27 and MEFV mutations in the development of sacroiliitis. The study group consisted of 256 FMF patients (male 128, female 128, mean age 27.2 ± 6.3 years). After evaluation of the medical records, 70 patients (27.4%) were determined to have one or more of musculoskeletal manifestations. Sacroiliitis was determined in 18 (32.7%) FMF patients. The frequency of sacroiliitis among all FMF patients was found to be 7%. HLA-B27 was 47% and 6.3% in FMF patients with and without sacroiliitis, respectively. The frequency of M694V mutations in FMF patients with sacroiliitis was 93.7%. Sacroiliitis may be seen more frequently in FMF patients than expected. HLA-B27 positivity and/or M694V mutation may play a role in the development of sacroiliitis and the severity of seronegative spondyloarthropathy.


Ankylosing spondylitis Familial Mediterranean fever HLA-B27 MEFV mutations Sacroiliitis 


  1. 1.
    Ben-Chetrit E, Levy M (1998) Familial Mediterranean fever. Lancet 351:659–664PubMedCrossRefGoogle Scholar
  2. 2.
    Langevitz P, Livneh A, Zemer D, Shemer Y, Pras M (1997) Seronegative spondyloarthropathy in familial Mediterranean fever. Semin Arthritis Rheum 27:67–72PubMedCrossRefGoogle Scholar
  3. 3.
    Keleş I, Aydın G, Tosun A, İnal E, Keleş H, Orkun S (2006) Familial Mediterranean fever and ankylosing spondylitis in a patient with juvenile idiopathic arthritis: a case report and review of the literature. Rheumatol Int 26:846–851PubMedCrossRefGoogle Scholar
  4. 4.
    Ozdolap Ş, Sarıkaya S, Aktunç E (2006) Familial Mediterranean fever and ankylosing spondylitis. Romatizma 21:31–33Google Scholar
  5. 5.
    Cefle A, Kamali S, Sayarlioglu M, Inanc M, Ocal L, Aral O et al (2005) A comparison of clinical findings of familial Mediterranean fever patients with and without amyloidosis. Rheumatol Int 25:442–6PubMedCrossRefGoogle Scholar
  6. 6.
    Yildiz M, Tunc SE, Sahin M, Okudan B, Aydin O, Suslu H, Ozbek M (2006) Evaluation of joints using Tc 99m-MDP bone scintigraphy in patients with familial Mediterranean fever: should bone scans be used for diagnosis and follow up? Rheumatol Int 26:220–223PubMedCrossRefGoogle Scholar
  7. 7.
    Livneh A, Pras M, Padeh S et al (1997) Criteria for the diagnosis of Familial Mediterranean fever. Arthritis Rheum 40:1879–1885PubMedCrossRefGoogle Scholar
  8. 8.
    Livneh A, Langevitz P, Zemer D, Zaks N, Kees S, Lidar T et al (1997) Establishment of a new set criteria for the classification of familial Mediterranean fever (FMF). In: Sohar E, Gafni J, Pras M (eds) Proceedings of the 1st international conference on familial Mediterranean fever. Freund, London and Tel Aviv, pp 203–208Google Scholar
  9. 9.
    van der Linden S, Valkenburg HA, Cats A (1984) Evaluation of diagnostic criteria for ankylosing spondylitis. A proposal for modification of the New York criteria. Arthritis Rheum 27:361–368PubMedCrossRefGoogle Scholar
  10. 10.
    Gofton J (1968) Report from the diagnostic criteria for ankylosing spondylitis. In: Bennett P, Wood PHN (eds) Population studies of the rheumatic diseases. Excerpta Medica, Amsterdam, pp 456–457Google Scholar
  11. 11.
    Song IH, Carrasco-Fernandez J, Rudwaleit M, Sieper J (2008) The diagnostic value of scintigraphy in assessing sacroiliitis in ankylosing spondylitis—a systemic literature research. Ann Rheum Dis doi: 10.1136/ard.2007.083089
  12. 12.
    Mimouni A, Magal N, Stoffman N, Shohat T, Minasian A, Krasnov M, et al. (2000) Familial Mediterranean fever: effects of genotype and ethnicity on inflammatory attacks and amyloidosis. Pediatrics 105: E70:Google Scholar
  13. 13.
    Ben-Chetrit E, Brautbar C, Rubinow A (1985) HLA antigens in Reiters’s syndrome in Israeli patients. J Rheumatol 12:964–966PubMedGoogle Scholar
  14. 14.
    Çelik S, Ozdoğan H, Hatemi G, Esen B, Seyahi E, Akcam Y (2007) Clinical, laboratory and radiological findings of patients with familial Mediterranean fever associated with ankylosing spondylitis (FMF-AS). Ann Rheum Dis 66(Suppl II):398Google Scholar
  15. 15.
    Yiğit S, Bağcı H, Ozkaya O, Ozdamar K, Cengiz K, Akpolat T (2008) MEFV mutations in patients with familial Mediterranean fever in the Black Sea region of Turkey. J Rheumatol 35:106–113PubMedGoogle Scholar
  16. 16.
    Olgun A, Akman S, Kurt I, Tüzün A, Kutluay T (2005) MEFV mutations in familial Mediterranean fever: association of M694V homozygosity with arthritis. Rheumatol Int 25:255–259PubMedCrossRefGoogle Scholar
  17. 17.
    Brik R, Shinawi M, Kasinetz L, Gershoni-Baruch R (2001) The musculoskeletal manifestations of familial Mediterranean fever in children genetically diagnosed with the disease. Arthritis Rheum 44:1416–1419PubMedCrossRefGoogle Scholar
  18. 18.
    Berkun Y, Ben-Chetrit E, Klar A, Ben-Chetrit E (2007) Peritoneal adhesions and intestinal obstructions in patients with familial Mediterranean fever—are they more frequent? Semin Arthritis Rheum 36:316–321PubMedCrossRefGoogle Scholar
  19. 19.
    Rabinovich E, Livneh A, Langevitz P, Brezniak N, Shinar E, Pras M et al (2005) Severe disease in patients with rheumatoid arthritis carrying a mutation in the Mediterranean fever gene. Ann Rheum Dis 64:1009–1014PubMedCrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2008

Authors and Affiliations

  • Timuçin Kaşifoğlu
    • 1
  • Cüneyt Çalışır
    • 1
    • 2
  • Döndü Ü. Cansu
    • 1
  • Cengiz Korkmaz
    • 1
    • 3
    Email author
  1. 1.Division of Rheumatology, Department of Internal MedicineEskişehir Osmangazi UniversityEskişehirTurkey
  2. 2.Department of RadiologyEskişehir Osmangazi UniversityEskişehirTurkey
  3. 3.Vişnelik Mah. Alifuat Güven CadEskişehirTurkey

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