Vascular Comorbidities in Familial Mediterranean Fever

Overview

Journal Rheumatol Int

Specialty Rheumatology

Date 2011 Mar 26

PMID 21437693

Citations 9

Authors

Z Birsin Ozcakar

Fatos Yalcinkaya

Affiliations

Soon will be listed here.

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.

Citing Articles

Central nervous system manifestations of monogenic autoinflammatory disorders and the neurotropic features of SARS-CoV-2: Drawing the parallels.

Renson T, Hamiwka L, Benseler S Front Pediatr. 2022; 10:931179.

PMID: 36034552 PMC: 9399631. DOI: 10.3389/fped.2022.931179.

Familial Mediterranean fever: What associations to screen for?.

Bouomrani S, Masmoudi I, Teber S Reumatologia. 2020; 58(3):150-154.

PMID: 32684647 PMC: 7362276. DOI: 10.5114/reum.2020.96688.

Familial Mediterranean fever and atherosclerosis in childhood and adolescence.

Vampertzi O, Papadopoulou-Legbelou K, Triantafyllou A, Douma S, Papadopoulou-Alataki E Rheumatol Int. 2019; 40(1):1-8.

PMID: 31705200 DOI: 10.1007/s00296-019-04457-8.

The Platelet-to-Lymphocyte Ratio as an Inflammatory Marker in Rheumatic Diseases.

Gasparyan A, Ayvazyan L, Mukanova U, Yessirkepov M, Kitas G Ann Lab Med. 2019; 39(4):345-357.

PMID: 30809980 PMC: 6400713. DOI: 10.3343/alm.2019.39.4.345.

Canakinumab: can it untie the Gordian knot of cardiovascular disease in patients with familial Mediterranean fever?.

Patoulias D Arch Med Sci Atheroscler Dis. 2019; 3:e96-e98.

PMID: 30775597 PMC: 6374570. DOI: 10.5114/amsad.2018.76866.

References

Centola M, Wood G, Frucht D, Galon J, Aringer M, Farrell C . The gene for familial Mediterranean fever, MEFV, is expressed in early leukocyte development and is regulated in response to inflammatory mediators. Blood. 2000; 95(10):3223-31. View

Ozcakar Z, Yalcinkaya F, Cakar N, Acar B, Kasapcopur O, Uguten D . MEFV mutations modify the clinical presentation of Henoch-Schönlein purpura. J Rheumatol. 2008; 35(12):2427-9. DOI: 10.3899/jrheum.080405. View

Bilginer Y, Ozaltin F, Basaran C, Duzova A, Besbas N, Topaloglu R . Evaluation of intima media thickness of the common and internal carotid arteries with inflammatory markers in familial Mediterranean fever as possible predictors for atherosclerosis. Rheumatol Int. 2008; 28(12):1211-6. DOI: 10.1007/s00296-008-0605-9. View

Rabinovich E, Shinar Y, Leiba M, Ehrenfeld M, Langevitz P, Livneh A . Common FMF alleles may predispose to development of Behcet's disease with increased risk for venous thrombosis. Scand J Rheumatol. 2007; 36(1):48-52. DOI: 10.1080/03009740600759639. View

Ugurlu S, Seyahi E, Cetinkaya F, Ozbakir F, Balci H, Ozdogan H . Intima-media thickening in patients with familial Mediterranean fever. Rheumatology (Oxford). 2009; 48(8):911-5. DOI: 10.1093/rheumatology/kep131. View

Tuglular S, Yalcinkaya F, Paydas S, Oner A, Utas C, Bozfakioglu S . A retrospective analysis for aetiology and clinical findings of 287 secondary amyloidosis cases in Turkey. Nephrol Dial Transplant. 2002; 17(11):2003-5. DOI: 10.1093/ndt/17.11.2003. View

Korkmaz C, Ozdogan H, Kasapcopur O, Yazici H . Acute phase response in familial Mediterranean fever. Ann Rheum Dis. 2002; 61(1):79-81. PMC: 1753891. DOI: 10.1136/ard.61.1.79. View

Cakar N, Yalcinkaya F, Ozkaya N, Tekin M, Akar N, Kocak H . Familial Mediterranean fever (FMF)-associated amyloidosis in childhood. Clinical features, course and outcome. Clin Exp Rheumatol. 2002; 19(5 Suppl 24):S63-7. View

Serrano R, Martinez M, Andres A, Morales J, Samartin R . Familial mediterranean fever and acute myocardial infarction secondary to coronary vasculitis. Histopathology. 1998; 33(2):163-7. DOI: 10.1046/j.1365-2559.1998.00462.x. View

10.

Turkmen A, Yildiz A, Erkoc R, Ecder T, Gorcin B, Turk S . Transplantation in renal amyloidosis. Clin Transplant. 1998; 12(5):375-8. View

11.

GOLDFINGER S . Colchicine for familial Mediterranean fever. N Engl J Med. 1972; 287(25):1302. DOI: 10.1056/NEJM197212212872514. View

12.

Peru H, Altun B, Dogan M, Kara F, Elmaci A, Oran B . The evaluation of carotid intima-media thickness in children with familial Mediterranean fever. Clin Rheumatol. 2007; 27(6):689-94. DOI: 10.1007/s10067-007-0764-1. View

13.

Akar S, Goktay Y, Akinci B, Tekis D, Biberoglu K, Birlik M . A case of familial Mediterranean fever and polyarteritis nodosa complicated by spontaneous perirenal and subcapsular hepatic hemorrhage requiring multiple arterial embolizations. Rheumatol Int. 2004; 25(1):60-4. DOI: 10.1007/s00296-003-0424-y. View

14.

Lachmann H, Sengul B, Yavuzsen T, Booth D, Booth S, Bybee A . Clinical and subclinical inflammation in patients with familial Mediterranean fever and in heterozygous carriers of MEFV mutations. Rheumatology (Oxford). 2006; 45(6):746-50. DOI: 10.1093/rheumatology/kei279. View

15.

Chae J, Aksentijevich I, Kastner D . Advances in the understanding of familial Mediterranean fever and possibilities for targeted therapy. Br J Haematol. 2009; 146(5):467-78. PMC: 2759843. DOI: 10.1111/j.1365-2141.2009.07733.x. View

16.

Livneh A, Langevitz P, Zemer D, Padeh S, Migdal A, SOHAR E . The changing face of familial Mediterranean fever. Semin Arthritis Rheum. 1996; 26(3):612-27. DOI: 10.1016/s0049-0172(96)80012-6. View

17.

Rozenbaum M, Naschitz J, Yudashkin M, Sabo E, Shaviv N, Gaitini L . Cardiovascular reactivity score for the assessment of dysautonomia in familial Mediterranean fever. Rheumatol Int. 2003; 24(3):147-52. DOI: 10.1007/s00296-003-0344-x. View

18.

Atagunduz P, Ergun T, Direskeneli H . MEFV mutations are increased in Behçet's disease (BD) and are associated with vascular involvement. Clin Exp Rheumatol. 2004; 21(4 Suppl 30):S35-7. View

19.

Gershoni-Baruch R, Broza Y, Brik R . Prevalence and significance of mutations in the familial Mediterranean fever gene in Henoch-Schönlein purpura. J Pediatr. 2003; 143(5):658-61. DOI: 10.1067/S0022-3476(03)00502-X. View

20.

Kallinich T, Haffner D, Niehues T, Huss K, Lainka E, Neudorf U . Colchicine use in children and adolescents with familial Mediterranean fever: literature review and consensus statement. Pediatrics. 2007; 119(2):e474-83. DOI: 10.1542/peds.2006-1434. View