Fibromuscular dysplasia: a rare disease that can mimic vasculitis

Abstract

Fibromuscular dysplasia (FMD) is an uncommon angiopathy that occurs mainly in young to middle-aged female individuals. It is an idiopathic, segmental, non-inflammatory and non-atherosclerotic vascular disease leading to stenosis of small- and medium-sized arteries. Clinical manifestations are determined by the artery involved, most commonly hypertension (renal artery) and stroke (carotid artery). When FMD affects multiple vascular beds, it may mimic a systemic vasculitis. Here, we present the case of a young female patient with FMD. The patient had a clinical history of bilateral internal carotid artery dissection that required surgical repair. Since a systemic vascular disease was suspected, abdominal angiography was done, showing evidence of a "string of beads" appearance involving the distal two-thirds of the right renal artery. This lesion is considered to be pathognomonic of the medial FMD that accounts for 70-95% of all cases of FMD. Two years later, a new magnetic resonance angiography confirmed the "string of beads" appearance of the middle to distal part of the right renal artery, with significant hemodynamic stenosis that was successfully dilated with percutaneous transluminal angioplasty.

Fibromuscular Dysplasia: A Rare Case of Renal Artery Stenosis Presenting with Stroke

“…The clinical presentation may vary from an asymptomatic condition to multiorgan disease including renovascular HT, AIS, cranial hemorrhage due to arterial dissection and abdominal pain or claudication. The disease can mimic vasculitis such as polyarteritis nodosa, giant cell arteritis or Takayasu arteritis (1,5). Our patient manifested with the symptoms of arterial insufficiency of brain and arterial occlusion of kidney.…”

“…Vital parameters were as follows; body temperature: 36. 5 Chest x-ray and electrocardiogram were normal. The urine analysis revealed no signs of protein and cellular elements.…”

“…The estimated incidence of childhood arterial ischemic stroke associated with renal arteriopathy and FMD is approximately 1:3000000 per year (2). This condition is typically associated with stenotic lesions in the renal, cerebral and cardiac arteries causing a characteristic "string of beads" appearance on angiography (1,(3)(4)(5). The most commonly described form of FMD is medial fibroplasia/dysplasia typically affecting young adult females with renal and cerebral vessel involvement, refractory hypertension (HT) and stroke.…”