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fibromuscular dysplasia

Etiology: - idiopathic 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: 1) dysplastic disease affecting medium-size & small arteries 2) disease can affect any layer of the arterial wall, medial fibroplasia is most common 3) 60-70% have renal vascular involvement 4) cerebrovascular involvement occurs in 30% of cases 5) stenotic lesions (renal artery stenosis) may limit or occlude blood flow & predispose to aneurysm formation or dissection 6) 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] a) 'string of beads' appearance b) represents alternating stenotic & aneurysmal regions of arteries - computed tomographic angiography [7] - sensitivity suboptimal for detecting distal renal artery stenosis, a common location for fibromuscular dysplasia [6] Management: 1) antiplatelet therapy for asymptomatic individuals [1,3] 2) percutaneous transluminal angioplasty for symptomatic patients - 2/3 of patients cured by angioplasty alone 3) surgery when angioplasty fails or when aneurysm or dissection is present [1,3]

Related

percutaneous transluminal coronary angioplasty (PTCA) renal artery stenosis (RAS)

General

dysplasia arterial disease

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 19. American College of Physicians, Philadelphia 1998, 2012, 2021
  2. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 898-99
  3. Olin JW, Sealove BA. Diagnosis, management, and future developments of fibromuscular dysplasia. J Vasc Surg. 2011 Mar;53(3):826-36.e1 PMID: 21236620
  4. 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: 29926082 https://jamanetwork.com/journals/jamacardiology/fullarticle/2685210
  5. 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: 24548843
  6. 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: 30648921
  7. Slovut DP, Olin JW.. Fibromuscular dysplasia N Engl J Med 2004;350:1862-71 PMID: 15115832
  8. NEJM Knowledge+