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Arthritis as the sole episodic manifestation of familial Mediterranean fever
Merav Lidar1, Ron Kedem, Adam Mor
1Heller Institute of Medical Research, Sheba Medical Center, Tel-Hashomer, Israel.
Objective:
To clinically and genetically characterize patients with familial Mediterranean fever (FMF) in whom arthritis constitutes the only manifestation, and to establish the most important features distinguishing FMF arthritis in such a setting from other forms of mono/oligo arthritides.
Methods:
The study population comprised 14 patients with episodes of arthritis as the only manifestation of FMF who nevertheless fulfilled the diagnostic criteria for FMF. The control group consisted of 28 patients with episodic mono/oligo arthritis of different disease entities (palindromic, reactive, inflammatory bowel disease, Reiter's, seronegative spondyloarthropathy, chronic juvenile, Behcet's, and gouty arthritis) who presented to the rheumatology clinic during the study period. Patients in both groups underwent clinical evaluation and donated blood for FMF gene analysis.
Results:
The study and control groups shared similar age and sex distribution and experienced the monoarthritic attacks at similar sites, usually the knee and ankle joint. The 2 groups differed significantly in features of arthritis (which were febrile and of short duration in FMF), family history of FMF, mutation analysis, and response to colchicine. These differences allowed the defining of a rule, which readily distinguishes FMF arthritis from other forms of episodic mono/oligo arthritis.
Conclusion:
The clinical, ethnic, and genetic features of recurrent monoarthritis of FMF are specific and may separate FMF from other entities with mono/oligo arthritis.
Insights
Familial Mediterranean fever (FMF) can present solely with arthritis. Key distinguishing features include fever, short duration, family history, specific gene mutations, and colchicine response, aiding differentiation from other episodic arthritis types.
Area of Science:
- Rheumatology
- Genetics
- Internal Medicine
Background:
- Familial Mediterranean fever (FMF) is an autoinflammatory disorder.
- Arthritis can be the sole presenting manifestation of FMF.
- Differentiating FMF arthritis from other mono/oligoarthritides is clinically important.
Purpose of the Study:
- To clinically and genetically characterize FMF patients with isolated arthritis.
- To identify features that distinguish FMF arthritis from other episodic mono/oligoarthritides.
Main Methods:
- Studied 14 FMF patients with arthritis as the only symptom.
- Compared them to 28 patients with other forms of episodic mono/oligoarthritis.
- Conducted clinical evaluations and FMF gene analysis for all participants.
Main Results:
- FMF arthritis attacks were typically febrile and short-lived, unlike controls.
- Significant differences observed in family history of FMF and mutation analysis.
- Response to colchicine was a key differentiator.
Conclusions:
- Clinical, ethnic, and genetic characteristics of FMF-related monoarthritis are distinct.
- These specific features allow for differentiation from other mono/oligoarthritis entities.
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