Summary

Fibromuscular dysplasia, a non-atherosclerotic and non-inflammatory disease of medium size arteries, commonly affects the renal and carotid arteries (the latter are dealt with in another article). It is more frequent in women from third to fifth decade and the genetic origin of the disease is proven in some cases. Once considered rare, for there are numerous asymptomatic cases, its prevalence in currently estimated around one percent. There are two distinct types of fibromuscular dysplasia: multi-focal (four out of five cases) and focal dysplasia. The most frequent presentation is hypertension in relation with renal artery involvement, which is amenable to percutaneous angioplasty. Fibromuscular dysplasia of digestive and iliac arteries is rare and usually asymptomatic. Patients with spontaneous coronary acute dissection commonly have dysplastic lesions affecting non-coronary arteries. Data from the ARCADIA registry show that the majority of patients with the condition have multisite fibromuscular dysplasia, i.e. lesions affecting two or more vascular beds.