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Fibromuscular dysplasia(FMDA)

MedGen UID:
4700
•Concept ID:
C0016052
•
Disease or Syndrome
Synonyms: Arterial fibromuscular dysplasia; Fibromuscular dysplasia of arteries
SNOMED CT: Fibromuscular hyperplasia of artery (783729004); Fibromuscular dysplasia of wall of artery (783729004); Fibromuscular hyperplasia (31653004); Fibromuscular dysplasia (31653004)
 
HPO: HP:0005313
Monarch Initiative: MONDO:0006761
OMIM®: 135580
Orphanet: ORPHA336

Definition

Fibromuscular dysplasia (FMDA) is a nonatherosclerotic, noninflammatory arterial disease that most commonly involves the renal and carotid arteries. The prevalence of symptomatic renal artery FMDA is about 4 in 1,000 and the prevalence of cervicocranial FMDA is about half of that. Histologic classification includes 3 main subtypes, intimal, medial, and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string of beads' appearance that is related to medial FMDA, and tubular and focal types, which are not clearly related to specific histologic lesions (summary by Plouin et al., 2007) [from OMIM]

Clinical features

From HPO
Renovascular hypertension
MedGen UID:
43786
•Concept ID:
C0020545
•
Disease or Syndrome
The presence of hypertension related to stenosis of the renal artery.
See: Feature record | Search on this feature
Fibromuscular dysplasia
MedGen UID:
4700
•Concept ID:
C0016052
•
Disease or Syndrome
Fibromuscular dysplasia (FMDA) is a nonatherosclerotic, noninflammatory arterial disease that most commonly involves the renal and carotid arteries. The prevalence of symptomatic renal artery FMDA is about 4 in 1,000 and the prevalence of cervicocranial FMDA is about half of that. Histologic classification includes 3 main subtypes, intimal, medial, and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string of beads' appearance that is related to medial FMDA, and tubular and focal types, which are not clearly related to specific histologic lesions (summary by Plouin et al., 2007)
See: Feature record | Search on this feature
Intermittent claudication
MedGen UID:
7115
•Concept ID:
C0021775
•
Disease or Syndrome
Intermittent claudication is a symptom of peripheral arterial occlusive disease. After having walked over a distance which is individually characteristic, the patients experience pain or cramps in the calves, feet or thighs which typically subsides on standing still.
See: Feature record | Search on this feature
Myocardial infarction
MedGen UID:
10150
•Concept ID:
C0027051
•
Disease or Syndrome
Necrosis of the myocardium caused by an obstruction of the blood supply to the heart and often associated with chest pain, shortness of breath, palpitations, and anxiety as well as characteristic EKG findings and elevation of serum markers including creatine kinase-MB fraction and troponin.
See: Feature record | Search on this feature
Stroke disorder
MedGen UID:
52522
•Concept ID:
C0038454
•
Disease or Syndrome
Sudden impairment of blood flow to a part of the brain due to occlusion or rupture of an artery to the brain.
See: Feature record | Search on this feature
Aortic dissection
MedGen UID:
83315
•Concept ID:
C0340643
•
Disease or Syndrome
Aortic dissection refers to a tear in the intimal layer of the aorta causing a separation between the intima and the medial layers of the aorta.
See: Feature record | Search on this feature
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Conditions with this feature

Fibromuscular dysplasia
MedGen UID:
4700
•Concept ID:
C0016052
•
Disease or Syndrome
Fibromuscular dysplasia (FMDA) is a nonatherosclerotic, noninflammatory arterial disease that most commonly involves the renal and carotid arteries. The prevalence of symptomatic renal artery FMDA is about 4 in 1,000 and the prevalence of cervicocranial FMDA is about half of that. Histologic classification includes 3 main subtypes, intimal, medial, and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string of beads' appearance that is related to medial FMDA, and tubular and focal types, which are not clearly related to specific histologic lesions (summary by Plouin et al., 2007)
See: Condition Record
Aneurysm, intracranial berry, 12
MedGen UID:
1684660
•Concept ID:
C5231484
•
Disease or Syndrome
Rupture of an intracranial aneurysm (IA), an outpouching or sac-like widening of a cerebral artery, leads to a subarachnoid hemorrhage (SAH), a sudden-onset disease that can lead to severe disability and death. Several risk factors such as smoking, hypertension, and excessive alcohol intake are associated with subarachnoid hemorrhage (summary by Krischek and Inoue, 2006).
See: Condition Record
Fibromuscular dysplasia, multifocal
MedGen UID:
1778238
•Concept ID:
C5543412
•
Disease or Syndrome
Multifocal fibromuscular dysplasia (FMDMF) is characterized histologically by medial fibroplasia and angiographically by multiple arterial stenoses with intervening mural dilations. Arterial tortuosity, macroaneurysms, dissections, and rupture may occur (summary by Richer et al., 2020).
See: Condition Record
Aneurysm, intracranial berry, 12
Fibromuscular dysplasia
Fibromuscular dysplasia, multifocal

Professional guidelines

PubMed

Cervical Artery Dissections: Etiopathogenesis and Management.
Keser Z, Chiang CC, Benson JC, Pezzini A, Lanzino G
Vasc Health Risk Manag 2022;18:685-700. Epub 2022 Sep 2 doi: 10.2147/VHRM.S362844. PMID: 36082197Free PMC Article
First International Consensus on the diagnosis and management of fibromuscular dysplasia.
Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, Bruno RM, de Leeuw P, Fendrikova-Mahlay N, Froehlich J, Ganesh SK, Gray BH, Jamison C, Januszewicz A, Jeunemaitre X, Kadian-Dodov D, Kim ES, Kovacic JC, Mace P, Morganti A, Sharma A, Southerland AM, Touzé E, van der Niepen P, Wang J, Weinberg I, Wilson S, Olin JW, Plouin PF
Vasc Med 2019 Apr;24(2):164-189. Epub 2019 Jan 16 doi: 10.1177/1358863X18821816. PMID: 30648921
Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association.
Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, Ganesh SK, Gulati R, Lindsay ME, Mieres JH, Naderi S, Shah S, Thaler DE, Tweet MS, Wood MJ; American Heart Association Council on Peripheral Vascular Disease; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Genomic and Precision Medicine; and Stroke Council
Circulation 2018 May 8;137(19):e523-e557. Epub 2018 Feb 22 doi: 10.1161/CIR.0000000000000564. PMID: 29472380Free PMC Article

Recent clinical studies

Etiology

Canadian Spontaneous Coronary Artery Dissection Cohort Study: 3-Year Outcomes.
Saw J, Starovoytov A, Aymong E, Inohara T, Alfadhel M, McAlister C, Samuel R, Grewal T, Parolis JA, Sheth T, So D, Minhas K, Brass N, Lavoie A, Bishop H, Lavi S, Pearce C, Renner S, Madan M, Welsh RC, McGrath BM, Vijayaraghavan R, Har B, Ibrahim R, Chaudhary P, Ganesh SK, Graham J, Matteau A, Martucci G, Ko DT, Humphries K, Mancini GJ
J Am Coll Cardiol 2022 Oct 25;80(17):1585-1597. doi: 10.1016/j.jacc.2022.08.759. PMID: 36265953
Renal artery stenosis.
Safian RD
Prog Cardiovasc Dis 2021 Mar-Apr;65:60-70. Epub 2021 Mar 18 doi: 10.1016/j.pcad.2021.03.003. PMID: 33745915
Carotid web: an occult mechanism of embolic stroke.
Mac Grory B, Emmer BJ, Roosendaal SD, Zagzag D, Yaghi S, Nossek E
J Neurol Neurosurg Psychiatry 2020 Dec;91(12):1283-1289. Epub 2020 Oct 1 doi: 10.1136/jnnp-2020-323938. PMID: 33004431
First International Consensus on the diagnosis and management of fibromuscular dysplasia.
Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, Bruno RM, de Leeuw P, Fendrikova-Mahlay N, Froehlich J, Ganesh SK, Gray BH, Jamison C, Januszewicz A, Jeunemaitre X, Kadian-Dodov D, Kim ES, Kovacic JC, Mace P, Morganti A, Sharma A, Southerland AM, Touzé E, van der Niepen P, Wang J, Weinberg I, Wilson S, Olin JW, Plouin PF
Vasc Med 2019 Apr;24(2):164-189. Epub 2019 Jan 16 doi: 10.1177/1358863X18821816. PMID: 30648921
Fibromuscular dysplasia (FMD).
Khoury MH, Gornik HL
Vasc Med 2017 Jun;22(3):248-252. Epub 2017 Apr 3 doi: 10.1177/1358863X17700716. PMID: 28367730

Diagnosis

Carotid web: an occult mechanism of embolic stroke.
Mac Grory B, Emmer BJ, Roosendaal SD, Zagzag D, Yaghi S, Nossek E
J Neurol Neurosurg Psychiatry 2020 Dec;91(12):1283-1289. Epub 2020 Oct 1 doi: 10.1136/jnnp-2020-323938. PMID: 33004431
Renovascular Hypertension.
Herrmann SM, Textor SC
Endocrinol Metab Clin North Am 2019 Dec;48(4):765-778. Epub 2019 Sep 19 doi: 10.1016/j.ecl.2019.08.007. PMID: 31655775Free PMC Article
Fibromuscular dysplasia (FMD).
Khoury MH, Gornik HL
Vasc Med 2017 Jun;22(3):248-252. Epub 2017 Apr 3 doi: 10.1177/1358863X17700716. PMID: 28367730
Renal fibromuscular dysplasia.
Mousa AY, Gill G
Semin Vasc Surg 2013 Dec;26(4):213-8. Epub 2014 Jun 13 doi: 10.1053/j.semvascsurg.2014.06.006. PMID: 25220329
Fibromuscular dysplasia.
Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X
Orphanet J Rare Dis 2007 Jun 7;2:28. doi: 10.1186/1750-1172-2-28. PMID: 17555581Free PMC Article

Therapy

Canadian Spontaneous Coronary Artery Dissection Cohort Study: 3-Year Outcomes.
Saw J, Starovoytov A, Aymong E, Inohara T, Alfadhel M, McAlister C, Samuel R, Grewal T, Parolis JA, Sheth T, So D, Minhas K, Brass N, Lavoie A, Bishop H, Lavi S, Pearce C, Renner S, Madan M, Welsh RC, McGrath BM, Vijayaraghavan R, Har B, Ibrahim R, Chaudhary P, Ganesh SK, Graham J, Matteau A, Martucci G, Ko DT, Humphries K, Mancini GJ
J Am Coll Cardiol 2022 Oct 25;80(17):1585-1597. doi: 10.1016/j.jacc.2022.08.759. PMID: 36265953
Renal artery stenosis.
Safian RD
Prog Cardiovasc Dis 2021 Mar-Apr;65:60-70. Epub 2021 Mar 18 doi: 10.1016/j.pcad.2021.03.003. PMID: 33745915
Carotid web: an occult mechanism of embolic stroke.
Mac Grory B, Emmer BJ, Roosendaal SD, Zagzag D, Yaghi S, Nossek E
J Neurol Neurosurg Psychiatry 2020 Dec;91(12):1283-1289. Epub 2020 Oct 1 doi: 10.1136/jnnp-2020-323938. PMID: 33004431
First International Consensus on the diagnosis and management of fibromuscular dysplasia.
Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, Bruno RM, de Leeuw P, Fendrikova-Mahlay N, Froehlich J, Ganesh SK, Gray BH, Jamison C, Januszewicz A, Jeunemaitre X, Kadian-Dodov D, Kim ES, Kovacic JC, Mace P, Morganti A, Sharma A, Southerland AM, Touzé E, van der Niepen P, Wang J, Weinberg I, Wilson S, Olin JW, Plouin PF
Vasc Med 2019 Apr;24(2):164-189. Epub 2019 Jan 16 doi: 10.1177/1358863X18821816. PMID: 30648921
Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association.
Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, Ganesh SK, Gulati R, Lindsay ME, Mieres JH, Naderi S, Shah S, Thaler DE, Tweet MS, Wood MJ; American Heart Association Council on Peripheral Vascular Disease; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Genomic and Precision Medicine; and Stroke Council
Circulation 2018 May 8;137(19):e523-e557. Epub 2018 Feb 22 doi: 10.1161/CIR.0000000000000564. PMID: 29472380Free PMC Article

Prognosis

Canadian Spontaneous Coronary Artery Dissection Cohort Study: 3-Year Outcomes.
Saw J, Starovoytov A, Aymong E, Inohara T, Alfadhel M, McAlister C, Samuel R, Grewal T, Parolis JA, Sheth T, So D, Minhas K, Brass N, Lavoie A, Bishop H, Lavi S, Pearce C, Renner S, Madan M, Welsh RC, McGrath BM, Vijayaraghavan R, Har B, Ibrahim R, Chaudhary P, Ganesh SK, Graham J, Matteau A, Martucci G, Ko DT, Humphries K, Mancini GJ
J Am Coll Cardiol 2022 Oct 25;80(17):1585-1597. doi: 10.1016/j.jacc.2022.08.759. PMID: 36265953
First International Consensus on the diagnosis and management of fibromuscular dysplasia.
Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, Bruno RM, de Leeuw P, Fendrikova-Mahlay N, Froehlich J, Ganesh SK, Gray BH, Jamison C, Januszewicz A, Jeunemaitre X, Kadian-Dodov D, Kim ES, Kovacic JC, Mace P, Morganti A, Sharma A, Southerland AM, Touzé E, van der Niepen P, Wang J, Weinberg I, Wilson S, Olin JW, Plouin PF
Vasc Med 2019 Apr;24(2):164-189. Epub 2019 Jan 16 doi: 10.1177/1358863X18821816. PMID: 30648921
Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association.
Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, Ganesh SK, Gulati R, Lindsay ME, Mieres JH, Naderi S, Shah S, Thaler DE, Tweet MS, Wood MJ; American Heart Association Council on Peripheral Vascular Disease; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Genomic and Precision Medicine; and Stroke Council
Circulation 2018 May 8;137(19):e523-e557. Epub 2018 Feb 22 doi: 10.1161/CIR.0000000000000564. PMID: 29472380Free PMC Article
Spontaneous Coronary Artery Dissection: Clinical Outcomes and Risk of Recurrence.
Saw J, Humphries K, Aymong E, Sedlak T, Prakash R, Starovoytov A, Mancini GBJ
J Am Coll Cardiol 2017 Aug 29;70(9):1148-1158. doi: 10.1016/j.jacc.2017.06.053. PMID: 28838364
Fibromuscular dysplasia (FMD).
Khoury MH, Gornik HL
Vasc Med 2017 Jun;22(3):248-252. Epub 2017 Apr 3 doi: 10.1177/1358863X17700716. PMID: 28367730

Clinical prediction guides

Canadian Spontaneous Coronary Artery Dissection Cohort Study: 3-Year Outcomes.
Saw J, Starovoytov A, Aymong E, Inohara T, Alfadhel M, McAlister C, Samuel R, Grewal T, Parolis JA, Sheth T, So D, Minhas K, Brass N, Lavoie A, Bishop H, Lavi S, Pearce C, Renner S, Madan M, Welsh RC, McGrath BM, Vijayaraghavan R, Har B, Ibrahim R, Chaudhary P, Ganesh SK, Graham J, Matteau A, Martucci G, Ko DT, Humphries K, Mancini GJ
J Am Coll Cardiol 2022 Oct 25;80(17):1585-1597. doi: 10.1016/j.jacc.2022.08.759. PMID: 36265953
First International Consensus on the diagnosis and management of fibromuscular dysplasia.
Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, Bruno RM, de Leeuw P, Fendrikova-Mahlay N, Froehlich J, Ganesh SK, Gray BH, Jamison C, Januszewicz A, Jeunemaitre X, Kadian-Dodov D, Kim ES, Kovacic JC, Mace P, Morganti A, Sharma A, Southerland AM, Touzé E, van der Niepen P, Wang J, Weinberg I, Wilson S, Olin JW, Plouin PF
Vasc Med 2019 Apr;24(2):164-189. Epub 2019 Jan 16 doi: 10.1177/1358863X18821816. PMID: 30648921
Spontaneous Coronary Artery Dissection: Current State of the Science: A Scientific Statement From the American Heart Association.
Hayes SN, Kim ESH, Saw J, Adlam D, Arslanian-Engoren C, Economy KE, Ganesh SK, Gulati R, Lindsay ME, Mieres JH, Naderi S, Shah S, Thaler DE, Tweet MS, Wood MJ; American Heart Association Council on Peripheral Vascular Disease; Council on Clinical Cardiology; Council on Cardiovascular and Stroke Nursing; Council on Genomic and Precision Medicine; and Stroke Council
Circulation 2018 May 8;137(19):e523-e557. Epub 2018 Feb 22 doi: 10.1161/CIR.0000000000000564. PMID: 29472380Free PMC Article
Spontaneous Coronary Artery Dissection: Clinical Outcomes and Risk of Recurrence.
Saw J, Humphries K, Aymong E, Sedlak T, Prakash R, Starovoytov A, Mancini GBJ
J Am Coll Cardiol 2017 Aug 29;70(9):1148-1158. doi: 10.1016/j.jacc.2017.06.053. PMID: 28838364
Fibromuscular dysplasia (FMD).
Khoury MH, Gornik HL
Vasc Med 2017 Jun;22(3):248-252. Epub 2017 Apr 3 doi: 10.1177/1358863X17700716. PMID: 28367730

Recent systematic reviews

Secondary Prevention and Rehabilitation for Spontaneous Coronary Artery Dissection: A Systematic Review.
Van Damme A, McDermott S, McMurtry S, Kung JY, Gyenes G, Norris C
Can J Cardiol 2023 Nov;39(11S):S395-S411. Epub 2023 Aug 19 doi: 10.1016/j.cjca.2023.08.013. PMID: 37604409
Optical coherence tomography in peripheral arterial disease: A systematic review.
Tung ET, Yim KHC, Li CL, Cheung CY, Chan YC
Int J Clin Pract 2021 Oct;75(10):e14628. Epub 2021 Jul 22 doi: 10.1111/ijcp.14628. PMID: 34258814
Systematic review and meta-analysis of the clinical characteristics and outcomes of spontanous coronary artery dissection.
Franke KB, Nerlekar N, Marshall H, Psaltis PJ
Int J Cardiol 2021 Jan 1;322:34-39. Epub 2020 Aug 28 doi: 10.1016/j.ijcard.2020.08.076. PMID: 32861717
Fibromuscular Dysplasia and Its Neurologic Manifestations: A Systematic Review.
Touzé E, Southerland AM, Boulanger M, Labeyrie PE, Azizi M, Bouatia-Naji N, Debette S, Gornik HL, Hussain SM, Jeunemaitre X, Joux J, Kirton A, Le Hello C, Majersik JJ, Mocco J, Persu A, Sharma A, Worrall BB, Olin JW, Plouin PF
JAMA Neurol 2019 Feb 1;76(2):217-226. doi: 10.1001/jamaneurol.2018.2848. PMID: 30285053
Efficacy of revascularization for renal artery stenosis caused by fibromuscular dysplasia: a systematic review and meta-analysis.
Trinquart L, Mounier-Vehier C, Sapoval M, Gagnon N, Plouin PF
Hypertension 2010 Sep;56(3):525-32. Epub 2010 Jul 12 doi: 10.1161/HYPERTENSIONAHA.110.152918. PMID: 20625080

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