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Published on: September 9, 2012
Familial mediterranean Fever and hypercoagulability
Oshrat E Tayer-Shifman1, Eldad Ben-Chetrit
1Rheumatology Unit, Division of Medicine, Hadassah-Hebrew University Medical Center. Jerusalem, Israel.
Abstract:
Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease which is characterized by recurrent attacks of fever and peritonitis, pleuritis, arthritis, or erysipelas-like skin disease. As such, FMF is a prototype of autoinflammatory diseases where genetic changes lead to acute inflammatory episodes. Systemic inflammation - in general - may increase procoagulant factors, and decrease natural anticoagulants and fibrinolytic activity. Therefore, it is anticipated to see more thrombotic events among FMF patients compared with healthy subjects. However, reviewing the current available literature and based upon our personal experience, thrombotic events related purely to FMF are very rare. Possible explanation for this discrepancy is that along with the procoagulant activity during FMF acute attacks, anticoagulant and fibrinolytic changes are also taking place. Colchicine which is the treatment of choice in FMF may also play a role in reducing inflammation thereby decreasing hypercoagulability.
Insights
Familial Mediterranean fever (FMF) is an autoinflammatory disease. Despite inflammation increasing clotting risk, thrombotic events are rare in FMF patients, possibly due to counterbalancing anticoagulant changes and colchicine treatment.
Area of Science:
- Rheumatology
- Genetics
- Hematology
Background:
- Familial Mediterranean fever (FMF) is an autosomal recessive autoinflammatory disease.
- FMF involves recurrent inflammatory episodes like fever, peritonitis, pleuritis, and arthritis.
- Systemic inflammation can promote a prothrombotic state by altering coagulation factors.
Purpose of the Study:
- To investigate the occurrence of thrombotic events in patients with Familial Mediterranean fever.
- To explore potential explanations for the observed frequency of thrombosis in FMF.
Main Methods:
- Literature review of current available studies on FMF and thrombosis.
- Analysis of personal clinical experience with FMF patients.
Main Results:
- Thrombotic events purely related to FMF are reported as very rare in the literature and clinical experience.
- Acute FMF attacks involve both procoagulant and anticoagulant/antifibrinolytic changes.
- Colchicine, the primary FMF treatment, may reduce inflammation and hypercoagulability.
Conclusions:
- The low incidence of thrombosis in FMF patients despite inflammatory triggers suggests complex hemostatic regulation.
- Concurrent anticoagulant and fibrinolytic activities may counteract the prothrombotic tendency during FMF attacks.
- Colchicine's anti-inflammatory effects likely contribute to a reduced thrombotic risk in FMF management.
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