Acute Pericarditis as the First Manifestation of Familial Mediterranean Fever: A Possible Relationship with Idiopathic Recurrent Pericarditis

Yoshioka, Katsunobu; Furumitsu, Yutaka; Sano, Tsuyoshi; Miyamoto, Terumasa; Agematsu, Kazunaga

doi:10.2169/internalmedicine.53.2064

Cited by 9 publications

(8 citation statements)

References 15 publications

(13 reference statements)

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“…While pericarditis typically presents simultaneously with other FMF symptoms,[ 8 ] it has been reported as the only manifestation of the attack. [ 6 7 11 13 14 15 ] In those cases, recurrent pericarditis which are refractory to the typical treatments raised the suspicion of FMF requiring further genetic studies to confirm the diagnosis.…”

Section: Pericardial Diseasesmentioning

confidence: 99%

Cardiac manifestations of Familial Mediterranean fever

2017

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Familial Mediterranean fever (FMF) is autoinflammatory disorder characterized by sporadic attacks of fever, peritonitis, pleuritis, and arthritis. It is mainly seen in patients from Mediterranean origins, but it is now reported more frequently in Europe and North America due to immigration. To analyze the data on the cardiovascular manifestations in FMF patients, we searched PubMed using the terms “Familial Mediterranean Fever” or “FMF” in combination with other key words including “cardiovascular diseases” “pericardial diseases” “atherosclerosis” “coronary artery diseases” “cardiomyopathy” “pulmonary hypertension” or “valvular diseases.” suggested several mechanisms to explain the cardiac involvements in FMF including the ongoing inflammation and the amyloid deposits in the heart and vessels' walls at the advanced stages of FMF. The course of these manifestations varies widely, but it can associate with poor prognosis in some cases such as with pulmonary hypertension. Interestingly, Colchicine, which is the cornerstone therapy of FMF, plays a vital role in treating and preventing some of these disorders. In this article, In this article, we will discuss the incidence, pathophysiology, and prognosis of the various cardiac manifestations affecting FMF patients.

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Section: Pericardial Diseasesmentioning

confidence: 99%

Cardiac manifestations of Familial Mediterranean fever

2017

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“…She was compound heterozygous for E148Q and L695A. Yoshioka et al [7] described a 56-year-old man who had a high fever, CRP level, and periodic episodes of acute pericarditis. The patient was treated with colchicine, became afebrile and CRP level decreased.…”

Section: Discussionmentioning

confidence: 99%

“…[3,4] Recurrent pericarditis as the initial finding has been reported in a few case with FMF. [5][6][7] Here, we report a patient with suspected FMF and recurrent pericarditis who responded to colchicine therapy.…”

Section: Introductionmentioning

confidence: 99%

Recurrent pericarditis caused by familial Mediterranean fever: A case report

Karaman¹

2021

Zeynep Kamil Med J

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Familial Mediterranean fever (FMF) is characterized by clinically recurrent fever at- tacks and inflammation associated with serositis. The Mediterranean fever gene is found in 16p13.3 and its mutations are known to lead to FMF. Here, we present a patient with FMF and recurrent pericarditis who responded to colchicine treatment. Therefore, regular follow-up of FMF patients who are mutation carriers is important in terms of cardiovascular risk.

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“…While most of the pericardial symptoms occur in conjunction with other FMF manifestations, solitary pericardial involvement has been described in the literature [24][25][26]. A diagnosis of FMF should be suspected in patients with recurrent episodes of pericarditis refractory to treatment, requiring further genetic analysis for the accurate diagnosis.…”

Section: Pericardial Diseasesmentioning

confidence: 99%

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

Malik

Shabbir

Nazir³

2020

Pulse

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Introduction: 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. Methods: 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. Results: 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. Conclusion: 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.

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Acute Pericarditis as the First Manifestation of Familial Mediterranean Fever: A Possible Relationship with Idiopathic Recurrent Pericarditis

Cited by 9 publications

References 15 publications

Cardiac manifestations of Familial Mediterranean fever

Cardiac manifestations of Familial Mediterranean fever

Recurrent pericarditis caused by familial Mediterranean fever: A case report

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

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