Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.

High Prevalence of Spondyloarthritis Among First Degree Relatives of Patients with Familial Mediterranean Fever: Further Evidence for a Link Between the Two Disorders.

Akar1,  Servet, Soysal2,  Ozgul, Solmaz2,  Dilek, Gerdan2,  Vedat, Onen1,  Fatos, Akkoc1,  Nurullah

Professor, Izmir, Turkey
MD, Izmir, Turkey


Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by recurrent attacks of fever and serositis. It is associated with the missense variations of Meditteranean fever (MEFV) gene. There is some evidence that spondyloarthritis (SpA) including ankylosing spondylitis (AS) is more prevalent in FMF patients. Moreover we and other groups showed M694V, the most penetrant MEFV variation, may be an additional susceptibility factor for AS. Therefore in the present study we investigated the prevalence of SpA and AS in first-degree relatives (FDRs) of FMF patients.


The FDRs (> 16 years old) of 201 consecutive unrelated FMF patients were invited to the outpatient clinic. They were examined according to a standard protocol to capture patients with SpA. Presence of inflammatory back pain (IBP) was judged according to the Calin and Berlin criteria. Standard pelvic X-rays of the sacroiliac joints (SIJ) were performed in all patients. The diagnosis of SpA and AS were made based on the European Spondyloarthritis Study Group (ESSG) and the modified New York (mNY) criteria, respectively.


The 201 probands had 1039 FDRs, who were over 16 years-old. Of them, 892 were alive. Ninety-six FDRs who could not be reached after at least three attempts and 84 FDRs with FMF were not included in the study. Of the remaining 712 FDRs, 319 agreed to participate of whom 233 (73%) reported back pain. IBP was present in 32 patients (13.7%) according to Calin criteria and in 20 patients (8.6%) according to Berlin criteria. Thirty-six FDRs were diagnosed as having SpA and five of them as having AS. One additional patient with AS was identified when the medical records of the 393 non-attending invitees were reviewed. The prevalence of SpA and AS among 712 FDRs were calculated as 5.1% and 0.8%, respectively. Based on a prevalence of 1.09% of SpA and 0.49% of AS found in a previous study conducted in our region, the risk ratio was estimated as 4.6 (95%CI; 2.9 to 7.4) for SpA and 1.7 (95%CI; 0.7 to 4.3) for AS in all the relatives. When only the parents (n=275) were considered, the risk ratios for SpA and AS, were 6.9 (95%CI; 4.1 to 11.9) and 2.94 (95% CI; 1.02 to 8.41). Furthermore, two patients with Behcet's syndrome, two patients with rheumatoid arthritis and one patient with un-differentiated arthritis were detected in the study population.


The higher frequency of SpA among the FDRs of the FMF patients and the higher prevalence of AS among the parents, are in line with the previously published studies which have reported high prevalence of sacroiliitis or SpA in FMF patients.

To cite this abstract, please use the following information:
Akar, Servet, Soysal, Ozgul, Solmaz, Dilek, Gerdan, Vedat, Onen, Fatos, Akkoc, Nurullah; High Prevalence of Spondyloarthritis Among First Degree Relatives of Patients with Familial Mediterranean Fever: Further Evidence for a Link Between the Two Disorders. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :541

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