fibromuscular dysplasia
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Etiology
Epidemiology
- women are affected more than men
- generally affects patients 15-30 years of age
- most likely cause of hypertension in an asymptomatic young person with normal laboratory tests[8]
- elderly patients with fibromuscular dysplasia may represent a more benign clinical phenotype[4]
Pathology
- dysplastic disease affecting medium-size & small arteries
- disease can affect any layer of the arterial wall, medial fibroplasia is most common
- 60-70% have renal vascular involvement
- cerebrovascular involvement occurs in 30% of cases
- stenotic lesions (renal artery stenosis) may limit or occlude blood flow & predispose to aneurysm formation or dissection
- not a result of atherosclerosis or inflammation
Clinical manifestations
- most common presentation is hypertension
Laboratory
- plasma renin activity elevated with renal vascular involvement
- other laboratory tests normal
Radiology
- duplex renal ultrasound
- may show asymmetric kidney size
- doppler may show renal artery stenosis
- renal artery CT angiography: (gold standard)[1]
- computed tomographic angiography[7]
- sensitivity suboptimal for detecting distal renal artery stenosis, a common location for fibromuscular dysplasia[6]
Management
- antiplatelet therapy for asymptomatic individuals[1][3]
- percutaneous transluminal angioplasty for symptomatic patients
- 2/3 of patients cured by angioplasty alone
- surgery when angioplasty fails or when aneurysm or dissection is present[1][3]
More general terms
Additional terms
References
- ↑ Jump up to: 1.0 1.1 1.2 1.3 Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 19. American College of Physicians, Philadelphia 1998, 2012, 2021
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 898-99
- ↑ Jump up to: 3.0 3.1 3.2 Olin JW, Sealove BA. Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg. 2011 Mar;53(3):826-36.e1 PMID: https://www.ncbi.nlm.nih.gov/pubmed/21236620
- ↑ Jump up to: 4.0 4.1 Bagh I, Olin JW, Froehlich JB et al Association of Multifocal Fibromuscular Dysplasia in Elderly Patients With a More Benign Clinical Phenotype. Data From the US Registry for Fibromuscular Dysplasia. JAMA Cardiol. Published online June 20, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29926082 https://jamanetwork.com/journals/jamacardiology/fullarticle/2685210
- ↑ Olin JW, Gornik HL, Bacharach JM et al Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association. Circulation. 2014 Mar 4;129(9):1048-78. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24548843
- ↑ Jump up to: 6.0 6.1 Gornik HL, Persu A, Adlam D First International Consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 2019 Apr;24(2):164-189 PMID: https://www.ncbi.nlm.nih.gov/pubmed/30648921
- ↑ Jump up to: 7.0 7.1 Slovut DP, Olin JW.. Fibromuscular dysplasia N Engl J Med 2004;350:1862-71 PMID: https://www.ncbi.nlm.nih.gov/pubmed/15115832
- ↑ Jump up to: 8.0 8.1 NEJM Knowledge+