Cardiovascular Sequelae and Genetics of Familial Mediterranean Fever: A Literature Review

Abstract

<b><i>Introduction:</i></b> Familial Mediterranean fever (FMF) is an autoinflammatory fever syndrome distinguished by recurrent attacks of spontaneous peritonitis, pleuritis, fever, and arthritis. It is specifically seen in the ethnic groups of Mediterranean origin, but sporadic cases have been reported in Eastern Europe and America due to migrations. There is a number of cardiac manifestations associated with FMF. <b><i>Methods:</i></b> Using PubMed as the search engine, the literature search was done for articles published between 1958 and 2020. To summarize the body of available evidence, a scoping review was carried out to find relevant articles and case reports in patients of FMF with cardiovascular manifestations. <b><i>Results:</i></b> In the literature, there is a number of mechanisms explaining the cause of cardiac involvement in FMF, including the subclinical inflammation and secondary (AA) amyloid deposition in the vessels and the myocardium. There is a variable and often spurious course of these manifestations and it can be associated with a poor prognosis such as an acute myocardial infarction. In FMF patients, polyarteritis nodosa and Henoch-Schönlein purpura are seen more significantly as compared to the general population with increased frequency of mutations in Mediterranean fever (MEFV) gene. Through unclear mechanisms, Behçet’s disease is associated with MEFV gene mutations and shares vascular manifestations with FMF. There is an interplay of IL-1 and MEFV gene, which impart an important role in inflammatory attacks of FMF. There is an intima-media thickening of blood vessels AA to persistent inflammation which can lead to atherosclerotic plaque formation resulting in atherosclerotic cardiovascular disease. <b><i>Conclusion:</i></b> FMF and its associated cardiovascular diseases are interlinked to 2 main mechanisms: subclinical atherosclerosis and amyloid deposition, and colchicine is the primary treatment of patients with FMF which shows the regression of amyloid deposits and prevents cardiovascular sequelae.

Effect of Maternal Familial Mediterranean Fever on Fetal Pulmonary Artery Acceleration/Ejection Time

“…Disruption of this protein, which is mostly expressed in neutrophils, causes an increase in interleukin 1(IL 1) and reactive oxygen species secretions and excessive inflammatory response of body itself. 4 The main cause of organ and tissue damage in FMF is free oxygen radicals with increased secretion from neutrophils. Subclinical inflammation, which continues in silent periods between acute attacks, may cause Amyloid-A accumulation called amyloidosis in kidney, liver and cardiac tissues.…”

Health-related quality of life in Egyptian patients with familial Mediterranean fever

“…FMF management main goal is to control and reduce the number of attacks and inflammation in-between the attacks and consequently ameliorating the patients' general QoL. Proper FMF management also prevents the longterm complications like amyloidosis and its effect on the kidneys (Malik et al, 2021).…”