Arterial Fibromuscular Dysplasia
Section snippets
PREVALENCE AND ANATOMIC DISTRIBUTION
Information about the prevalence of FMD in patients with vascular disease is incomplete. In the hypertensive population, high blood pressure may be attributable to renovascular FMD in fewer than 2% of the patients.13 In patients with renovascular hypertension, FMD is the underlying cause in 20 to 50%.14, 15, 16 FMD of the renal artery has also been found in healthy renal transplant donors.13 In an autopsy study of 819 consecutive examinations, 9 instances of renovascular FMD were found, an
PATHOLOGIC CLASSIFICATION AND RADIOLOGIC APPEARANCE
A pathologic classification of FMD was proposed by Harrison and McCormack22 in 1971 and was revised by Stanley and colleagues23 in 1975. The histologic classification is based on the predominant site of dysplasia in the arterial wall: the intima, media, or adventitia (Fig. 2). Hence, three main types of FMD have been identified—intimal fibroplasia, medial FMD, and periarterial or periadventitial fibroplasia. Lesions involving the medial layer of the artery may be further subdivided into medial
DIFFERENTIAL DIAGNOSIS
The differential diagnosis of FMD includes arteriosclerosis, inflammatory vascular diseases such as Takayasu's arteritis, and vascular lesions of neurofibromatosis. Certain types of hereditary connective tissue diseases, such as Ehlers-Danlos syndrome, may angiographically mimic the aneurysmal type of FMD.27
FMD is a histologic diagnosis; however, the diagnosis can be made with a high degree of accuracy on the basis of the angiographic appearance. Classic string-of-beads stenoses are consistent
NATURAL HISTORY
Progression of FMD has been confirmed on repeated angiograms in a substantial number of patients with renovascular disease. Meaney and co-workers32 reported that during an observation period of 6 months to 10 years, renovascular disease progressed in 36% of patients with atherosclerosis but in only 16% of those with FMD. In a Mayo Clinic series, during a mean observation period of 3 years, FMD of the renal arteries progressed in 35% of the patients.33 Progression was more common in older
PATHOGENESIS
The cause of FMD remains unknown. Several hypotheses on the pathogenesis have been proposed. The most important concepts are (1) the humoral hypothesis, in which involvement of the female sex hormones has been suggested; (2) the mechanical hypothesis, in which the importance of trauma or repeated microtrauma has been emphasized; (3) the genetic hypothesis; and (4) the hypothesis of ischemia of the blood vessel wall. It is unlikely that all subtypes of FMD have the same underlying cause.
CLINICAL MANIFESTATIONS AND MANAGEMENT OF FMD
The clinical manifestations of FMD are determined by the arteries involved, the degree of vascular occlusion, and the presence or absence of a collateral circulation. Hence, the patients may be asymptomatic or may have signs and symptoms of vascular occlusive disease. Renovascular hypertension, transient ischemic attacks, and stroke are the most common clinical manifestations.
Coarctation of the Aorta
Coarctation of the thoracic aorta is 4 or 5 times more frequent in male than in female patients. In contrast, coarctation of the abdominal aorta is evenly distributed between the sexes or, as in FMD, has a slight preponderance in females.10, 166, 167, 168 An association of FMD of the renal arteries and coarctation of the abdominal aorta has been observed in seven patients.6, 10, 166, 167 In five of them, histologic findings in the renal arteries or the coarctation (or both) were compatible with
REFERENCES (172)
- et al.
Hypertension in unilateral renal disease
J Urol
(1938) - et al.
Fibromuscular disease of the renal arteries
Med Clin North Am
(May 1977) Fibromuscular hyperplasia: report of a case with involvement of multiple arteries
Am J Med
(1970)- et al.
A pathologic-arteriographic correlation of renal arterial disease
Am Heart J
(1966) - et al.
Isolated fibromuscular dysplasia of the coronary arteries with spontaneous dissection and myocardial infarction
Hum Pathol
(1987) - et al.
Renovascular hypertension: a rare cardiovascular manifestation of the Ehlers-Danlos syndrome
Mayo Clin Proc
(1987) - et al.
Serial renal function and angiographic observations in idiopathic fibrous and fibromuscular stenoses of the renal arteries
Am J Cardiol
(1972) - et al.
Natural history of atherosclerotic and fibrous renal artery disease: clinical implications
Am J Kidney Dis
(1985) - et al.
Spontaneous reversal of hypertension caused by fibromuscular dysplasia
J Pediatr
(1982) - et al.
Cigarette smoking and renovascular hypertension
Lancet
(1983)
Traumatic occlusion in fibromuscular dysplasia of the carotid artery
Surg Neurol
Ultrastructural characteristics of experimental arterial medial fibroplasia induced by vasa vasorum occlusion
J Surg Res
Immunologic considerations in renovascular hypertension
Urol Clin North Am
Renovascular hypertension: mechanisms, natural history and treatment
Am J Cardiol
Predictive value and changes of renin secretion in hypertensive patients with unilateral renovascular disease undergoing successful renal angioplasty
Am J Med
Renovascular hypertension: does the renal vein renin ratio predict operative results?
J Urol
Renal venous renin activity: enhancement of sensitivity of lateralization by sodium depletion
Am J Cardiol
Renal infarction due to renal artery dysplasia with dissection: report of a case in a normotensive patient
Am J Med
Treatment of renovascular hypertension with percutaneous transluminal dilatation of a renal-artery stenosis
Lancet
Long-term experience in percutaneous transluminal dilatation of renal artery stenosis
Am J Med
Acute and chronic effects of the angiotensin-converting enzyme inhibitor captopril in severe hypertension
Am J Cardiol
Surgical treatment of renovascular hypertension in the pediatric patient
J Urol
Results of surgical treatment of renovascular hypertension
J Urol
Surgical management of branch renal artery disease: in situ versus extracorporeal methods of repair
J Urol
Fibromuscular hyperplasia: extension of the disease and therapeutic outcome; results of the University Hospital Zurich Cooperative Study on Fibromuscular Hyperplasia
Nephron
Obstructive lesions of the renal artery associated with remediable hypertension (abstract)
Am J Pathol
Fibromuscular hyperplasia of the internal carotid artery: report of a case
Ann Surg
Fibromuscular dysplasia and the brain: observations on angiographic, clinical and genetic characteristics
Stroke
Extrarenaler Gefässbefall bei fibromuskulär bedingter renovaskulärer Hypertonie
Klin Wochenschr
Fibromuscular hyperplasia of the left axillary artery
Arch Surg
Extrarenal fibromuscular hyperplasia
Am J Surg
Fibromuscular hyperplasia and dissecting aneurysm of the hepatic artery
Arch Pathol
Fibromuscular hyperplasia of the abdominal aorta
J Cardiovasc Surg (Torino)
Arterial dysplasia: an important surgical lesion
Arch Surg
Angiographic appearance of the renal vein in a case of fibromuscular hyperplasia of the artery
Radiology
Clinical characteristics of renovascular hypertension
JAMA
Renal venous renin activity in various forms of curable renal hypertension
Clin Nephrol
Percutaneous transluminal renal angioplasty in renovascular hypertension due to atheroma or fibromuscular dysplasia
N Engl J Med
Arterial fibromuscular dysplasia studied at autopsy (abstract)
Am J Clin Pathol
Cephalic arterial fibromuscular dysplasia
Radiology
Cerebral ischemic events in patients with carotid artery fibromuscular dysplasia
Arch Neurol
Renal and extrarenal arterial fibromuscular hyperplasia with hypertension
NZ Med J
Pathologic classification of renal arterial disease in renovascular hypertension
Mayo Clin Proc
Arterial fibrodysplasia: histopathologic character and current etiologic concepts
Arch Surg
Angiographic spectrum of cervical and intracranial fibromuscular dysplasia
Stroke
Vasculitis
Neurofibromatose et lésions vasculaires
Schweiz Med Wochenschr
Vascular lesions causing hypertension in neurofibromatosis
N Engl J Med
Neurofibromatosis of the renal artery
Br J Radiol
Natural history of renal arterial disease
Radiology
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Present address: Division of Cardiology, University Hospital, Basel, Switzerland.
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Present address: Ochsner Clinic, New Orleans, Louisiana.