Fibromuscular Dysplasia

An idiopathic, segmental, nonatheromatous disease of the musculature of arterial walls, leading to STENOSIS of small and medium-sized arteries. There is true proliferation of SMOOTH MUSCLE CELLS and fibrous tissue. Fibromuscular dysplasia lesions are smooth stenosis and occur most often in the renal and carotid arteries. They may also occur in other peripheral arteries of the extremity.

Also Known As:

Networked: 298 relevant articles (13 outcomes, 18 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Renal Artery Obstruction (Renal Artery Stenosis)
2.	Hypertension (High Blood Pressure)
3.	Renovascular Hypertension (Goldblatt Syndrome)
4.	Pathologic Constriction (Stenosis)
5.	Atherosclerotic Plaque (Atheroma)

Experts

1.	Zeller, Thomas: 5 articles (01/2014 - 12/2004)
2.	Sandmann, W: 3 articles (07/2010 - 12/2002)
3.	Voiculescu, A: 3 articles (07/2010 - 12/2002)
4.	Cohen, José E: 2 articles (11/2014 - 08/2007)
5.	Kalva, Sanjeeva P: 2 articles (06/2014 - 08/2013)
6.	Gulati, Rajiv: 2 articles (05/2014 - 01/2014)
7.	Grabitz, Klaus: 2 articles (04/2014 - 02/2003)
8.	Saw, Jacqueline: 2 articles (04/2014 - 01/2014)
9.	Rastan, Aljoscha: 2 articles (01/2014 - 11/2009)
10.	Zeller, T: 2 articles (03/2011 - 08/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fibromuscular Dysplasia:

1.	ReninIBA 06/01/2015 - "1D.11: RENAL MICROVASCULATURE AND RENIN SECRETION IN HUMANS WITH MULTIFOCAL RENAL ARTERY FIBROMUSCULAR DYSPLASIA." 03/01/1990 - "Tests showed all of them to have fibromuscular dysplasia stenosis of the main renal artery and significant lateralization of renin levels. " 10/01/1983 - "On examination, her blood pressure was 220/140 mmHg, and plasma renin activity was 4.2 ng/ml/hr. The selective renal arteriogram showed fibromuscular dysplasia of the left main renal artery, and a diagnosis of renovascular hypertension in the solitary kidney with an aberrant artery was made. " 03/01/1982 - "arteriosclerosis (AS) or fibromuscular dysplasia (FMD) and to the value of Renal: Systemic Renin Indices (RSRI) available in 30 cases. " 02/10/1984 - "Percutaneous transluminal angioplasty (PTA) of renal artery stenosis has proved highly efficacious, particularly in patients with lateralizing renal vein renin ratios, fibromuscular dysplasia, and atherosclerotic lesions confined to one renal artery without involvement of the aortic wall surrounding the renal ostium. "
2.	Progesterone Receptors (Progesterone Receptor)IBA 01/01/2015 - "Intranuclear expression of progesterone receptors in smooth muscle cells of renovascular fibromuscular dysplasia: a pilot study."
3.	FibrinIBA 08/01/1976 - "In this study of three fatal cases of pheochromocytoma the small pulmonary arteries were narrowed by a variety of chronic and acute processes which included medial hypertrophy and fibrosis, endothelial proliferation and fibrosis, and endothelial cellular edema; within the sinus node, atrioventricular (A-V) node and His bundle of all three cases there was focal degeneration and fibrosis similar to that also observed throughout the ventricular myocardium; and in addition to the focal narrowing of many small coronary arteries produced by medial hypertrophy, intimal fibrosis, and fibromuscular dysplasia, there were also focal aggregations of platelets clogging the lumen and occasionally mixed with a varying amount of fibrin. "
4.	alpha 1-Antitrypsin (alpha 1 Antitrypsin)IBA 09/01/1996 - "Recent studies have suggested that a deficiency of alpha 1-antitrypsin may be a genetic risk factor for the development of intracranial aneurysms and arterial fibromuscular dysplasia. "
5.	glucuronyl glucosamine glycan sulfate (Vessel)IBA 08/01/2013 - "Some arteriopathies such as fibromuscular dysplasia and tortuosity make a vessel predisposed to dissection. " 03/01/2013 - "Fibromuscular dysplasia may also produce the same pattern and should be distinguished from vessel tortuosity. " 08/01/2007 - "In one, histological examination of a vessel biopsy revealed medial fibromuscular dysplasia. " 03/01/1995 - "This case suggests that the frequency of fibromuscular dysplasia as a cause for "idiopathic" spontaneous carotid artery dissection may be higher than previously recognized and that recurrent embolization may occur in the setting of chronic dissection due to redissection of the previously involved vessel." 11/01/1993 - "Some arteriopathies such as fibromuscular dysplasia and tortuosity make a vessel predisposed to dissection."
6.	CollagenIBA 03/01/2006 - "Fibromuscular dysplasia and abundant collagen deposition were the predominant features seen on histology. " 07/01/2003 - "Adventitial fibromuscular dysplasia (AFMD) consists of proliferation of smooth muscle cells and collagen fibers in the adventitia of blood vessels. " 06/01/2014 - "Imaging findings overlap with inflammatory vasculitis, collagen vascular disease, and fibromuscular dysplasia. " 09/01/2003 - "Uncommon arteriopathies encompass a diverse range of diseases, including inherited collagen vascular disorders such as Marfan syndrome, Ehlers-Danlos Type IV, and pseudoxanthoma elasticum; vasculitides, including Takayasu's arteritis, extracranial giant cell arteritis, and Behçet's disease; neurofibromatosis type 1; intimal fibromuscular dysplasia; unusual bacterial and viral infections; and drug-induced arteriopathies. " 10/01/2014 - "Disordered collagen within the muscularis (identified using picrosirius red histochemistry) was confined to fibromuscular dysplasia; fibrocellular smooth muscle intimal proliferation within parenchymal cerebral arteries was confirmed using smooth muscle actin immunohistochemistry in the Moyamoya case; the patient with Ehlers-Danlos type IV showed aneurysm formation and eccentric intimal thickening of circle of Willis vessels but no obvious abnormalities of the muscularis; and neurofibromatosis I cases showed extensive leptomeningeal smaller-caliber arterial disease that particularly affected the spinal cord. "
7.	Phenobarbital (Luminal)FDA Link 11/01/2009 - "The etiology of this renovascular disease is most of the time due to a fibromuscular dysplasia (FMD), which causes a noninflammatory intimal-medial fibroplasia leading to luminal compromise. " 07/01/2004 - "Fibromuscular dysplasia (FMD) is a noninflammatory arteriopathy characterized by intimal, medial, and/or adventitial fibroplasias leading to luminal compromise and aneurysm formation. " 06/01/1994 - "Typical causes of renovascular hypertension include intramural atherosclerotic lesions of the main renal arteries or their branches and fibromuscular dysplasia of the renal arterial wall with luminal narrowing. " 03/01/1996 - "Conventional arteriograms were evaluated for dissection site, evidence of fibromuscular dysplasia, luminal stenosis or occlusion, and pseudoaneurysm formation. " 07/01/1992 - "Fibromuscular dysplasia is characterized by medical fibrosis with or without smooth muscle cell hyperplasia and may produce luminal impingement with severe turbulence. "
8.	Oral ContraceptivesIBA 08/31/1991 - "Associations with fibromuscular dysplasia, arterial hypertension and the use of oral contraceptives have been reported. " 11/01/1987 - "Dissection was spontaneous in 8 patients, occurred after neck manipulation in 2 and after a potential minor injury to the neck in 3. Six had a history of common migraine, 4 were using oral contraceptives at the time of dissection, and 3 had fibromuscular dysplasia. " 01/01/1987 - "Pathogenetic factors (lupus erythematodes, endocarditis, fibromuscular dysplasia) and risk factors (cigarette smoking, oral contraceptives) were found in 5 patients whereas in 2 cases the etiology was not determinable. " 07/01/1993 - "Other significant etiologic differences included fibromuscular dysplasia, mitral valve prolapse, and use of oral contraceptives occurring more frequently in the younger group, and ischemic heart disease occurring more frequently in the older group. " 01/01/1983 - " The etiology of the stenosis was probably atherosclerosis, except in 1 case which was due to fibromuscular dysplasia, 1 to postradiation angiopathy, and 2 related to oral contraceptives. "
9.	Mesenteric vascular diseaseIBA 03/01/2015 - "Both CTA and MRA are suitable for the assessment of patients with suspected chronic mesenteric ischemia, allowing to evaluate the degree of atherosclerotic steno-occlusive disease and the existence of collateral circulation, as well as other nonatherosclerotic vascular pathologies such as fibromuscular dysplasia and median arcuate ligament syndrome. " 11/01/2014 - "Such disorders include fibromuscular dysplasia, median arcuate ligament syndrome, the renal nutcracker syndrome, and some forms of acute and chronic mesenteric ischemia (embolic and thrombotic). " 01/01/2010 - "We report the first known case of chronic mesenteric ischemia from fibromuscular dysplasia. " 01/01/2010 - "A rare cause of chronic mesenteric ischemia from fibromuscular dysplasia: a case report." 08/01/2013 - "Chronic mesenteric ischemia is almost always caused by atherosclerotic disease, with rare causes including fibromuscular dysplasia and vasculitis. "
10.	Cocaine (Cocaine HCl)IBA 09/01/2007 - "More intriguingly, the case suggests a possible relationship between in utero cocaine exposure and the development of fibromuscular dysplasia in the child." 09/01/2007 - "Fibromuscular dysplasia in association with intrauterine cocaine exposure." 01/01/2014 - "RAT has both cardiogenic etiology (arrhythmia, endocarditis, valvular heart disease, coronary disease) and noncardiogenic etiology (renal artery injury and/or instrumentation, fibromuscular dysplasia, coagulation disorders, homocystinuria, cocaine intake). " 01/01/2015 - "Non-cardiovascular conditions were connective tissue disorders, fibromuscular dysplasia, Ehlers-Danlos syndrome, Marfan's syndrome, sarcoidosis, Crohn's disease, polycystic kidney disease, rheumatoid arthritis, vasculitis including giant cell arteritis, polyarteritis nodosa and Takayasu's disease, cocaine use, early or premature labor. " 04/01/1996 - "The reported risk factors of this age group are cardiopathies, hypertension, smoking, hypercholesterolemia, reduction of anticoagulant proteins, hypercoagulable states, antiphospholipid antibodies primary syndrome, antiphospholipid antibodies secondary syndrome, some hemoglobinopathies, hyperviscosity syndromes, vasculitis, collagen vascular diseases, fibromuscular dysplasia, arterial dissections, migraine, myopathy encephalopathy lactic acidosis stroke like episodes, homocystinuria, familial amyloid angiopathy, microangiopathy with retinopathy encephalopathy and deafness, systemic lupus erythematosus, use of cocaine, traumas or manipulations of neck, AIDS. "

Therapies and Procedures

1.	Angioplasty (Angioplasty, Transluminal) 08/04/1983 - "After successful angioplasty, blood pressure was reduced to normal or improved in 93 per cent of the patients with fibromuscular dysplasia and in 84 per cent of the patients with atheromatous disease. " 04/01/2015 - "Surgical management of fibromuscular dysplasia is now less common because of the better efficacy of percutaneous transluminal angioplasty. " 06/01/2005 - "Although renal angioplasty is of proven benefit in fibromuscular dysplasia, randomized trials in atherosclerotic RAS have not shown any advantage for revascularization over medical therapy in terms of blood pressure control or renal function preservation. " 04/01/1992 - "To test whether duplex scanning results after intervention correlate with clinical outcome, we reviewed the studies on nine patients with renal artery fibromuscular dysplasia who had been treated by either angioplasty or surgery. " 12/01/2015 - "Long-term follow-up for percutaneous transluminal angioplasty in renal artery fibromuscular dysplasia."
2.	Balloon Angioplasty 10/01/2014 - "Short- and mid-term results of balloon angioplasty for renal artery fibromuscular dysplasia." 11/01/2009 - "Diffuse fibromuscular dysplasia successfully treated with scoring balloon angioplasty in a 3-year-old boy." 11/01/2005 - "Regarding "Cutting balloon angioplasty of renal fibromuscular dysplasia: a word of caution"." 02/01/2002 - "Treatment of renal artery fibromuscular dysplasia with balloon angioplasty: a prospective follow-up study." 02/01/1999 - "Conventional balloon angioplasty of non-ostial RAS caused by fibromuscular dysplasia with a high technical and functional success rate may be the treatment of choice. "
3.	Transplants (Transplant) 12/01/2015 - "Spontaneous LIMA graft dissection in a patient with fibromuscular dysplasia." 06/01/2012 - "Herein, we report a case of renal transplant using a kidney with fibromuscular dysplasia that had higher glomerular filtration rate than the normal side. " 06/01/2012 - "Successful transplant of a kidney with fibromuscular dysplasia having higher glomerular filtration rate than the contralateral kidney." 08/01/1999 - "Some complex cases of fibromuscular dysplasia may necessitate patch insertion or excision and graft interposition." 11/01/1989 - "The role of several factors that have been suggested as being of etiologic importance in renovascular fibromuscular dysplasia was examined in a case-control study of 33 patients with angiographically demonstrated fibromuscular dysplasia and 61 renal transplant donor control subjects with normal renal arteries. "
4.	Balloon Dilation 03/01/1985 - "Gruntzig balloon dilation for fibromuscular dysplasia of the internal carotid arteries." 03/01/2004 - "The most common clinical manifestation of fibromuscular dysplasia is hypertension, which can frequently be cured or significantly improved with percutaneous balloon dilation. "
5.	Denervation 01/01/2013 - "Renal denervation in fibromuscular dysplasia." 01/01/2013 - "Renal denervation offers novel treatment opportunities in patients with resistant hypertension, including cases with rare causes (fibromuscular dysplasia), warranting further investigation. "