Disclosures � Advisor: Innovein, inc Fibromuscular Dysplasia Diagnosis, Treatment and Surveillance Marlene Grenon, MD Department of Surgery April 2017 4/6/2017 4/6/2017 40 year-old woman referred for arm and leg 40 year-old woman referred for arm and leg weakness with exertion and chronic fatigue. Is weakness with exertion and chronic fatigue. Is this PAD or FMD? this PAD or FMD? This is not FMD! FMD mimic: Standing waves - vasospasm vs. artifact 3 4 Sharma A, Gornik H. Circ Cardiovasc Interv. 2012 . Sharma A, Gornik H. Circ Cardiovasc Interv. 2012 . 1 4/6/2017 [ADD PRESENTATION TITLE: INSERT TAB > HEADER & FOOTER > NOTES AND HANDOUTS]
Plan: Review Relevant Concepts in FMD Misconception #1 Management All coronary, carotid, and renal artery disease is caused by atherosclerosis. � Approach FMD with emphasis on contemporary data from the FMD Registry • Presentation • Suggested Surveillance • Treatment � Address misconceptions Olin JW, et al. Circulation 2012;125:3182. 5 4/6/2017 6 Olin et al, Circ 2014 4/6/2017 Misconception #1 Fibromuscular Dysplasia is… All coronary, carotid, and renal artery disease is caused by � Non-atherosclerotic, non-inflammatory arterial disease (not veins) atherosclerosis. � Affects medium-sized vessels � FMD can cause renal, visceral, cerebrovascular, extremity, and • Also described in almost every vascular bed coronary disease. � First described by Leadbetter and Burkland in 1938 � Many patients have few or no atherosclerotic risk factors. • 5 yo boy with severe HTN 2ry to an occluded renal artery (intra-arterial mass of smooth muscl e) � Whereas atherosclerosis occurs at the origin or proximal portion of the vessel, FMD occurs in the mid and distal part of the artery. � Arterial stenosis, beading, dissection and aneurysm Brachial Artery FMD Yoshimuta, T. et al. Circulation 2008;117:2542. Olin et al, Circ 2014; Yoshimuta, T. et al. Circulation 2008;117:2542. 7 4/6/2017 8 Leadbetter WF and Burkland CE. Hypertension in unilateral renal artery J. Urol 1938 4/6/2017 2 4/6/2017 [ADD PRESENTATION TITLE: INSERT TAB > HEADER & FOOTER > NOTES AND HANDOUTS]
Epidemiology Prevalence � Initially thought that renal FMD >> carotid FMD � Rare disease (National � Disease of women [9:1] � Currently a paradigm shift prompted from US Registry for FMD Organization for Rare Diseases) • Women present with classic � Most common: renal arteries = carotid (+/- vertebral arteries) • <200,000 in US residents signs and symptoms • Meta-analysis ~4% of potential • Men more likely present with kidney donor population visceral involvement • Consecutive cerebral angiograms: • Men have a 2-fold increase in 0.3-3.2% prevalence of arterial aneurysm and dissection O’Connor and Gornik, JAHA 2014, Lorenx et al, Clin J Am Soc Nephrol 2010; Blondin et al, Eur J Radiol 2010; Cragg et al, Rad 1989; McKenie et al, J Vasc Interv 9 Radiol 2013. 4/6/2017 10 Olin et al, Circulation 2012 4/6/2017 Etiology… Theory but uncertainty Nomenclature is changing remains � No etiologic genes identified � Historically, classified histopathologically into categories based on the dominant arterial layer � 60% of cases are thought familial Etiology • Media, intima, adventitia � Overlapping features with CTD � And the composition of the arterial lesion Congenital • Loeys-Dietz syndrome Injury • Collagen deposition: fibroplasia • Ehlers-Danlos (vascular type) Hormonal • Hyperplasia of smooth muscle cells � Likely gene-environment interactions CTD • Smoking Medial fibroplasia-> “string of bead” • Estrogen Intimal and adventitial fibroplasia-> tubular and focal stenosis 11 Rushton, Arch Intern Med 1980; Olin et al, Circ 2012 4/6/2017 12 4/6/2017 3 4/6/2017 [ADD PRESENTATION TITLE: INSERT TAB > HEADER & FOOTER > NOTES AND HANDOUTS]
Nomenclature is changing European Consensus (2012) � Multifocal, tubular and unifocal FMD Multifocal Tubular Atypical Unifocal 13 4/6/2017 14 Persu et al, J Hypert 2014 4/6/2017 Multifocal (Medial Fibroplasia) American Heart Association (2014) � Medial fibroplasia most common angiographic variant ~90% of cases � Collagen deposition in vessel media • Internal elastic lamina and adventitia generally spared � Sequential areas of stenosis (webs) followed by dilatation • “String of beads” • “String of pearls” • “Stack of coins” • “Sausage links” With permission from H. Gornik; 1 Dr. Seymour Rosen in Kumar: Robbins and Cotran Pathologic Basis of Disease. 8 th Edition. 15 Olin et al, Circ 2014; O’Connor and Gornik JAHA 2014 4/6/2017 16 4/6/2017 2 Casanegra AC, et al. SVM 2010. 4 4/6/2017 [ADD PRESENTATION TITLE: INSERT TAB > HEADER & FOOTER > NOTES AND HANDOUTS]
Unifocal (Intimal Fibroplasia) When to consider the diagnosis � Classic Symptoms: � < 10% of cases • Hypertension � Vascular intima involved • Headache, usually migraine type • Collagen deposits in the intima There is an average • Internal elastic lamina is abnormal • Pulsatile tinnitus (“swoosh”) delay from the time of � Variable angiographic appearance the first symptoms or • Dizziness sign to diagnosis of • Focal, severe concentric stenosis • Cervical bruit 4-9 years. • Longer, tubular lesions: can mimic large • Neck pain vessel vasculitis (Takayasu arteritis) � Much more common in pediatric FMD cases � The signs and symptoms are broader � May present with aggressive, multi-vessel syndrome 17 4/6/2017 18 Savard et al, Circ 2012; Olin et al, Circ 2012 4/6/2017 With permission from H. Gornik; Symptoms US Registry 2012 (447 patients) Misconception #2 The most common presentation for carotid FMD is TIA or stroke. Hypertension 64% Hemispheric TIA 9% Headache 52% Post-prandial abdo pain 9% Stroke 8% Pulsatile tinnitus 28% Claudication 7% Dizziness 26% Amaurosis fugax 5% Cervical bruit 22% Weight loss 5% Neck pain 22% Horner syndrome 5% Tinnitus 19% Renal artery dissection 3% Chest Pain/SOB 16% Azotemia 2% Flank/abdo pain 16% Aneurysms 14% Myocardial infarction 2% Cervical artery dissection 12% Mesenteric ischemia 1% Epigastric bruit 9% No signs/symptoms 6% 19 O’Connor and Gornik, JAHA 2014; Olin et al. Circulation 2012 4/6/2017 20 Presentation Title and/or Sub Brand Name Here 4/6/2017 5 4/6/2017 [ADD PRESENTATION TITLE: INSERT TAB > HEADER & FOOTER > NOTES AND HANDOUTS]
Misconception #2 Aneurysms and Dissections in FMD In Registry, presented as % The most common presentation for carotid FMD is TIA or stroke. � Although TIA, stroke, and cervical dissection can occur with carotid FMD, the most common presentations represent nonspecific symptoms. • Headaches, dizziness, light-headedness, and pulsatile tinnitus � Carotid FMD can also be asymptomatic and detected incidentally via imaging for another reason or when a cervical bruit is appreciated. Carotid artery FMD with intracranial aneurysm 21 Presentation Title and/or Sub Brand Name Here 4/6/2017 22 Olin et al. Circulation 2012 4/6/2017 Summary for Presentation: Differential Diagnosis to Consider Who is the Typical FMD Patient? � 91% of patients are female � Average age of diagnosis 52 years � Standing Waves or Stationary Waves • 1 st FMD related symptom @ age 47 • 5 year delay in diagnosis! � Atherosclerosis � Most common symptoms/signs: � Vasculitis • Hypertension • Headache, usually migraine type � Segmental Arterial Mediolysis • Pulsatile tinnitus ( “ swoosh ” ) � Ehlers-Danlos syndrome • Dizziness • Cervical bruit � Neurofibromatosis type I • Neck pain � Williams syndrome � 20% of FMD patients have had an arterial dissection; 17% have had an aneurysm � Reversible cerebral vasoconstriction syndrome � FMD disease location • Most common: renal arteries = carotid +/- vertebral arteries � Median arcuate ligament syndrome • Less common: mesenteric, external iliac arteries • Uncommon: brachial arteries, intracranial FMD, coronary arteries • More than 1/3 patients have more than 1 vascular bed involved Olin JW, et al. Circulation 23 Olin et al. Circulation 2012 4/6/2017 2012;125:3182. 6 4/6/2017 [ADD PRESENTATION TITLE: INSERT TAB > HEADER & FOOTER > NOTES AND HANDOUTS]
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