Fibromuscular Dysplasia – Underrecognized Vasculopathy with Female Preponderance

Fibromuscular dysplasia (FMD) is a non-atherosclerotic, non-inflammatory vascular disease with strong female preponderance. However the diagnosis of the disease is low. FMD affects medium-sized muscular arteries, predominantly renal arteries, but equally carotid and vertebral arteries. It leads to artery stenosis, occlusion, aneurysm or dissection. The clinical picture depends on the affected blood vessels. It is most commonly recognized as a cause of hypertension in young female patients. In the case of involvement of cranial and cervical arteries, the clinical picture is mostly nonspecific, with headache, migraine and tinnitus. In registries, females had more frequent classical symptoms of cerebrovascular FMD like pulsatile tinnitus, cervical bruit and neck pain, and males have more frequently visceral affection (abdominal pain, renal impairment and renal infarction). Also, man had more frequently more sever clinical presentation like arterial dissection or arterial aneurysm. At the age of diagnosis males are younger compared to females. Large cohort studies showed the association of FMD with female sex, migraine, and intracranial aneurysm. FMD is an under-recognized vascular disease, more prevalent in females, but exhibiting more sever clinical presentation in males.