From the Research
The first diagnostic criteria for Familial Mediterranean Fever (FMF) were the Tel Hashomer criteria, developed in 1997, which remain the most widely used and accepted criteria for diagnosis, as supported by the study published in 1997 1.
Diagnostic Criteria
The Tel Hashomer criteria are divided into major and minor criteria. Major criteria include:
- Recurrent febrile episodes with peritonitis, pleuritis, or synovitis
- Amyloidosis of AA type without predisposing disease
- Favorable response to colchicine treatment Minor criteria include:
- Recurrent febrile episodes
- Erysipelas-like erythema
- FMF in a first-degree relative A definite diagnosis requires two major criteria or one major and two minor criteria, while a probable diagnosis requires one major and one minor criterion.
Clinical Presentation and Genetic Testing
These criteria were developed before genetic testing became widely available and rely primarily on clinical presentation, making them useful in clinical practice, especially in regions where genetic testing is not readily accessible, as noted in the study published in 2025 2.
Importance of Early Diagnosis
The diagnosis of FMF is important to establish early, as treatment with colchicine (typically 0.5-2 mg daily) can prevent recurrent attacks and the development of amyloidosis, which is the most serious complication of untreated FMF, as highlighted in the study published in 2020 3.
Treatment Options
For colchicine-resistant patients, IL-1 antagonists like anakinra and canakinumab show promise, although their long-term benefits require further investigation, as discussed in the study published in 2022 4.
Key Points
- The Tel Hashomer criteria are the first and most widely used diagnostic criteria for FMF
- Early diagnosis and treatment with colchicine can prevent recurrent attacks and amyloidosis
- IL-1 antagonists are a treatment option for colchicine-resistant patients
- Genetic testing can support diagnosis, but clinical presentation remains a key factor, as noted in the study published in 2015 5