Cardiac disease in familial Mediterranean fever

Rheumatol Int. 2018 Jan;38(1):51-58. doi: 10.1007/s00296-017-3853-8. Epub 2017 Oct 20.


Familial Mediterranean fever (FMF) is an autoinflammatory disease manifested by inflammatory attacks of peritonitis, pleuritis, pericarditis accompanied by fever and arthritis. Mutations of MEFV gene results in pyrin dysfunction, which causes uncontrolled interleukin-1 beta production and triggers the inflammatory attacks. Inflammation persists even during attack-free periods in one-third of the FMF patients. Findings of elevated proinflammatory cytokine patterns during remission as well as inflammatory attacks indicate the continuous subclinical disease activity and inflammation. Chronic inflammation was thought to be related to the cardiovascular risk in FMF patients. Main cardiac manifestations reported in FMF are pericarditis, idiopathic recurrent pericarditis, pericardiac tamponade, coronary heart disease and abnormal cardiovascular reactivity. Cardiac involvement in FMF may often be related to secondary AA amyloidosis. Deposition of amyloid may lead to cardiovascular morbidity and mortality in FMF patients. Associations of several vasculitic disorders such as Immunoglobulin A-associated vasculitis, polyarteritis nodosa and Behcet's disease are common in FMF. Appropriate prophylactic treatment with colchicine is recommended to prevent from cardiovascular risks. For those resistant to colchicine, IL-1 inhibitor agents can be used. Associated vasculitis should be treated with immunosuppressive agents. This review article aims to compile information about cardiac disease in FMF and refer to recent studies on the topic.

Keywords: Amyloidosis; Atherosclerosis; Cardiovascular disease; Familial Mediterranean fever; Heart diseases; Inflammation; Vasculitis.

Publication types

  • Review

MeSH terms

  • Amyloidosis / etiology*
  • Cardiovascular Diseases / etiology*
  • Colchicine / therapeutic use
  • Familial Mediterranean Fever / complications*
  • Familial Mediterranean Fever / drug therapy
  • Genotype
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Inflammation / etiology
  • Phenotype
  • Tubulin Modulators / therapeutic use
  • Vasculitis / drug therapy
  • Vasculitis / etiology


  • Immunosuppressive Agents
  • Tubulin Modulators
  • Colchicine