Comparison of Clinical and Demographic Features of FMF with Sacroiliitis Patients with FMF and Axial Spondyloarthritis Patients

Abstract

Objectives Familial Mediterranean fever (FMF) is the most common autoinflammatory disease, characterised by recurrent fever and serositis attacks lasting 1–3 days. Musculoskeletal involvement is the second most common manifestation in FMF patients. Sacroiliitis is another musculoskeletal involvement; as there is no spinal involvement, this is called FMF with sacroiliitis. This study was designed to investigate the clinical, demographic and genetic features of FMF in sacroiliitis patients and to compare them with axial SpA and FMF patients.

Materials and Methods Forty-two FMF with sacroiliitis patients, 100 axial SpA patients and 100 FMF patients were recruited, and their demographic characteristics were recorded. Evidence of sacroiliitis was confirmed by sacroiliac joint MRI, and patients were examined for arthritis and enthesitis. MEFV gene mutations, HLA B27 positivity and ESR and CRP results were compared.

Results In the FMF with sacroiliitis group, the M694V mutation was detected in 59.5% of patients. FMF with sacroiliitis patients were largely (83.3%) negative for HLA B27. The frequency of enthesitis was similar between FMF with sacroiliitis and axial SpA, and the frequency of arthritis was higher in axial SpA patients. Inflammatory markers (ESR and CRP) were statistically higher in FMF with sacroiliitis patients compared with axial SpA and FMF patients.

Conclusion When all three groups were compared, the M694V mutation was more common, HLA B27 was largely negative and inflammatory markers were higher in the FMF with sacroiliitis group. FMF should be included in the differential diagnosis of sacroiliitis for managing treatment correctly and preventing complications.

Zusammenfassung

Ziele Familiäres Mittelmeerfieber ( FMF) ist die häufigste autoinflammatorische Erkrankung, die sich durch wiederholte, 1–3 Tage andauernde Fieber- und Serositisanfälle auszeichnet. Die muskuloskelettale Beteiligung ist die zweithäufigste Manifestation bei Patienten mit FMF. Eine weitere muskuloskelettale Beteiligung ist die Sacroiliitis und wird wegen fehlender Wirbelsäulenbeteiligung als FMF-assoziierte Sacroiliitis/Spondyloarthritis bezeichnet. Diese Studie wurde entwickelt, um die klinischen, demografischen und genetischen Merkmale von FMF bei Sakroiliitis-Patienten zu untersuchen und mit axialen SpA- und FMF-Patienten zu vergleichen.

Materialen und Methoden 42 Sacroiliitis-Patienten mit FMF, 100 axiale SpA-Patienten und 100 FMF-Patienten wurden eingeschlossen und deren demographische Merkmale aufgezeichnet. Der Nachweis einer Sakroiliitis wurde durch MRT des Iliosakralgelenks bestätigt, und die Patienten wurden auf Arthritis und Enthesitis untersucht. MEFV-Genmutationen, HLA B27-Positivität sowie ESR- und CRP-Ergebnisse wurden verglichen.

Ergebnisse In der FMF mit Sakroiliitis-Gruppe wurde die M694V-Mutation bei 59,5% der Patienten nachgewiesen. Sacroiliitis-Patienten mit FMF waren überwiegend (83,3%) negativ für HLA B27. Die Häufigkeit der Enthesitis war zwischen FMF mit Sakroiliitis und axialem SpA ähnlich, und die Häufigkeit von Arthritis war bei Patienten mit axialem SpA höher. Die Entzündungsmarker (ESR undCRP) waren für Sacroiliitis-Patienten mit FMF im Vergleich zu Patienten mit axialer SpA und FMF statistisch höher.

Fazit Nach Vergleich dieser drei Gruppen trat die M694V-Mutation häufiger auf, HLA B27 war weitgehend negativ und die Entzündungsmarker waren in der Sacroiliitis-Gruppe mit FMF höher. Das FMF ist eine mögliche Differential diagnose der Sakroillitis und sollte als solche, bei entsprechendem Kontext, berücksichtigt werden.

Key words

FMF with sacroiliitis - axial SpA - FMF - MEFV - HLA B27

Schlüsselwörter

FMF mit Sakroiliitis - axialem SpA - FMF - MEFV - HLA B27

Publikationsverlauf

Artikel online veröffentlicht:
15. Oktober 2020

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