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Catheter-based Endovascular Angioplasty for Fibrosing Mediastinitis-associated Pulmonary Vein Stenosis
Published on: August 26, 2025
Vascular comorbidities in familial Mediterranean fever
Z Birsin Ozçakar1, Fatoş Yalçınkaya
1Division of Pediatric Nephrology, School of Medicine, Ankara University, Çınar Sitesi 5. Blok No: 62, Ümitköy, 06530, Ankara, Turkey.
Abstract:
Familial Mediterranean fever (FMF) is a common hereditary autoinflammatory disorder characterized by recurrent febrile attacks and polyserositis. The MEditerranean FeVer (MEFV) gene missense mutations altering the structure and function of pyrin protein play a significant role in the pathophysiology of the disease. Mutated pyrin is associated with the loss of delicate control of the inflammatory pathways, which results in a prolonged or augmented inflammation that predisposes these patients and carriers of the MEFV mutation to a pro-inflammatory state. This increased inflammation might lead to susceptibility to vascular comorbidities in FMF patients and even in carriers. In this review, we aim to discuss the vascular comorbidities seen in FMF patients. For this purpose, a thorough search was done in Web sites such as Pubmed, Web of Science, Scopus and Google Scholar, and the most relevant articles and case reports were evaluated. It seems that various vasculitides and the emerging problem of atherosclerosis have increasingly been recognized in these patients and, on the other hand, cardiac amyloidosis appears as a rare but devastating complication of FMF. Future studies will shed light on the unknown aspects of the emerging vascular problems in patients with FMF.
Insights
Familial Mediterranean Fever (FMF), an autoinflammatory disorder caused by MEFV gene mutations, increases the risk of vascular issues. This review explores these associated vascular comorbidities in FMF patients.
Area of Science:
- Genetics and Immunology
- Rheumatology
- Vascular Medicine
Background:
- Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disorder.
- It is characterized by recurrent fevers and polyserositis, linked to MEFV gene mutations affecting pyrin protein.
- These mutations cause uncontrolled inflammation, potentially increasing vascular risks.
Purpose of the Study:
- To review and discuss vascular comorbidities in patients with Familial Mediterranean Fever.
- To highlight the link between FMF, its genetic basis, and cardiovascular health.
Main Methods:
- A comprehensive literature search was conducted using major scientific databases (PubMed, Web of Science, Scopus, Google Scholar).
- Relevant articles and case reports on FMF and vascular complications were evaluated.
Main Results:
- Various vasculitides and atherosclerosis are increasingly recognized in FMF patients.
- Cardiac amyloidosis is a rare but severe complication.
- FMF patients and even carriers exhibit a pro-inflammatory state linked to vascular risks.
Conclusions:
- FMF is associated with a spectrum of vascular comorbidities, including vasculitis, atherosclerosis, and cardiac amyloidosis.
- Understanding these risks is crucial for managing FMF patients.
- Further research is needed to elucidate the full scope of vascular problems in FMF.
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