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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
Management of Renovascular Hypertension.
Smith A, Gaba RC, Bui JT, Minocha J
Tech Vasc Interv Radiol 2016 Sep;19(3):211-7. Epub 2016 Jun 3 doi: 10.1053/j.tvir.2016.06.006. PMID: 27641455

Recent clinical studies

Etiology

Spontaneous coronary artery dissection.
Méndez-Eirín E, Suárez-Ouréns Y, Rodríguez-Fernández JÁ
Rev Clin Esp (Barc) 2021 May;221(5):297-305. Epub 2021 Mar 31 doi: 10.1016/j.rceng.2020.11.003. PMID: 33998517
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
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.
Begelman SM, Olin JW
Curr Opin Rheumatol 2000 Jan;12(1):41-7. doi: 10.1097/00002281-200001000-00007. PMID: 10647953

Diagnosis

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.
Slovut DP, Olin JW
N Engl J Med 2004 Apr 29;350(18):1862-71. doi: 10.1056/NEJMra032393. PMID: 15115832
Fibromuscular dysplasia.
Begelman SM, Olin JW
Curr Opin Rheumatol 2000 Jan;12(1):41-7. doi: 10.1097/00002281-200001000-00007. PMID: 10647953

Therapy

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
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
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
Anti-hypertensive treatment in peripheral artery disease.
Tsioufis C, Andrikou I, Siasos G, Filis K, Tousoulis D
Curr Opin Pharmacol 2018 Apr;39:35-42. Epub 2018 Feb 22 doi: 10.1016/j.coph.2018.01.009. PMID: 29459079
Management of Renovascular Hypertension.
Smith A, Gaba RC, Bui JT, Minocha J
Tech Vasc Interv Radiol 2016 Sep;19(3):211-7. Epub 2016 Jun 3 doi: 10.1053/j.tvir.2016.06.006. PMID: 27641455

Prognosis

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
Management of Renovascular Hypertension.
Smith A, Gaba RC, Bui JT, Minocha J
Tech Vasc Interv Radiol 2016 Sep;19(3):211-7. Epub 2016 Jun 3 doi: 10.1053/j.tvir.2016.06.006. PMID: 27641455
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.
Sperati CJ, Aggarwal N, Arepally A, Atta MG
Kidney Int 2009 Feb;75(3):333-6. Epub 2008 Jun 4 doi: 10.1038/ki.2008.232. PMID: 18528320

Clinical prediction guides

Prevalence and outcomes of select rare vascular conditions in females: A descriptive review.
Sorber R, Bowen CJ, Radomski SN, Shalhub S
Semin Vasc Surg 2023 Dec;36(4):571-578. Epub 2023 Oct 7 doi: 10.1053/j.semvascsurg.2023.10.003. PMID: 38030331
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
Management of Renovascular Hypertension.
Smith A, Gaba RC, Bui JT, Minocha J
Tech Vasc Interv Radiol 2016 Sep;19(3):211-7. Epub 2016 Jun 3 doi: 10.1053/j.tvir.2016.06.006. PMID: 27641455
Coronary artery manifestations of fibromuscular dysplasia.
Michelis KC, Olin JW, Kadian-Dodov D, d'Escamard V, Kovacic JC
J Am Coll Cardiol 2014 Sep 9;64(10):1033-46. doi: 10.1016/j.jacc.2014.07.014. PMID: 25190240Free PMC Article

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
Factors associated with recurrent spontaneous coronary artery dissection: a systematic review and meta-analysis.
Chi G, Najafi H, Montazerin SM, Lee JJ
Coron Artery Dis 2022 Nov 1;33(7):566-573. Epub 2022 Jul 22 doi: 10.1097/MCA.0000000000001168. PMID: 35866498
Progress in aorta and peripheral cardiovascular disease research.
Mazzolai L, Alatri A, Rivière AB, De Carlo M, Heiss C, Espinola-Klein C, Schlager O, Sillesen H, Staub D, Rodriguez-Palomares JF, Verstraeten A, Aboyans V; WG on aorta and peripheral vascular diseases
Cardiovasc Res 2021 Jul 27;117(9):2045-2053. doi: 10.1093/cvr/cvab144. PMID: 33892507Free PMC Article
Ruptured Superior Mesenteric Artery Aneurysm due to Fibromuscular Dysplasia: A Rare Vascular Presentation in a Patient with Schizophrenia.
Silvestri V, Sapienza P, Ossola P, Grande R, Brachini G, Sterpetti AV, Mingoli A
Ann Vasc Surg 2019 Jul;58:384.e5-384.e8. Epub 2019 Feb 13 doi: 10.1016/j.avsg.2018.11.030. PMID: 30769066
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

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