Abstract
Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease, mainly affecting populations originating from the Eastern Mediterranean region. Behçet’s Disease (BD) is grouped in polygenic autoinflammatory diseases. It is a systemic vasculitis that affects all types and sizes of blood vessels. The aim of this article is to shed light on similarities and differences between FMF and BD. BD is frequently reported along the ancient Silk Road, extending from the Far East to the Mediterranean basin. Several studies have searched for the association between FMF and BD. FMF is caused by mutations of the MEditerranean FeVer (MEFV) gene while an increased frequency of MEFV mutations is reported in BD patients. Although BD and FMF share some epidemiological and pathophysiological features, there are distinct clinical characteristics of these nosological entities. Mucocutaneous manifestations, especially recurrent oral ulcers, are the most common symptom in BD patients whereas fever accompanied by serosal inflammation is the main clinical presentation in FMF patients.
Highlights
Familial Mediterranean fever (FMF) and Behçet’s disease (BD) are autoinflammatory diseases common in the Mediterranean region
Familial Mediterranean Fever (FMF) is the most common monogenic autoinflammatory disease, mainly affecting populations originating from the Eastern Mediterranean region
FMF is caused by mutations of the MEditerranean FeVer (MEFV) gene while an increased frequency of MEFV mutations is reported in Behçet’s Disease (BD) patients
Summary
Familial Mediterranean fever (FMF) and Behçet’s disease (BD) are autoinflammatory diseases common in the Mediterranean region. They do share some features and may co-occur in one patient. FMF is the most common autoinflammatory disease with autosomal recessive inheritance It mainly affects populations originating from the Mediterranean region, predominantly Armenians, Turks, Jews, and Arabs [1]. Endoplasmic reticulum aminopeptidase 1 (ERAP1) is cause pyrin inflammasome activation, leading to an another enzyme that plays a pathogenic role in BD. It increase in caspase-1 activation and interleukin 1β
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