Cardiac disease in familial Mediterranean fever

Abstract

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.

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

“…syndromes [TRAPS] and systemic-onset juvenile idiopathic arthritis [Still's disease]) suggested a common etiological pathway (213)(214)(215)(216)(217)(218)(219)(220)(221)(222)(223)(224). Likely, relapsing pericarditis presents family aggregation in 10% of patients (225).…”

Cardiovascular disease risk assessment in patients with familial Mediterranean fever related renal amyloidosis

“…Renal AA amyloidosis with end-stage renal disease is strongly associated with excess mortality in individuals with FMF, especially due to cardiac complications 6 – 8 . Increased cardiovascular disease (CVD) risk in patients with FMF may also be related to chronic inflammation 7 , 9 , 10 . CVD is also the main cause of morbidity and mortality in patients with glomerulonephropathies (GN) 11 .…”

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

“…Colchicine is considered as the first line treatment option in patients with FMF, with potential preventive action against cardiovascular disease, partially due to improvement in endothelial function [10, 11]. However, it is ineffective or not tolerated in a significant proportion of those patients (5–10%).…”