Vascular comorbidities in familial Mediterranean fever

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.