The United States registry for fibromuscular dysplasia

Results in the first 447 patients

Jeffrey W. Olin, James Froehlich, Xiaokui Gu, J. Michael Bacharach, Kim Eagle, Bruce H. Gray, Michael R. Jaff, Esther S H Kim, Pam MacE, Alan H. Matsumoto, Robert D. McBane, Eva Kline-Rogers, Christopher J. White, Heather L. Gornik

Research output: Contribution to journalArticle

247 Citations (Scopus)

Abstract

Background-Fibromuscular dysplasia (FMD), a noninflammatory disease of medium-size arteries, may lead to stenosis, occlusion, dissection, and/or aneurysm. There has been little progress in understanding the epidemiology, pathogenesis, and outcomes since its first description in 1938. Methods and Results-Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from 9 US sites. Vascular beds were imaged selectively based on clinical presentation and local practice. The majority of patients were female (91%) with a mean age at diagnosis of 51.9 (SD 13.4 years; range, 5-83 years). Hypertension, headache, and pulsatile tinnitus were the most common presenting symptoms of the disease. Self-reported family history of stroke (53.5%), aneurysm (23.5%), and sudden death (19.8%) were common, but FMD in first-or second-degree relatives was reported only in 7.3%. FMD was identified in the renal artery in 294 patients, extracranial carotid arteries in 251 patients, and vertebral arteries in 82 patients. A past or presenting history of vascular events were common: 19.2% of patients had a transient ischemic attack or stroke, 19.7% had experienced arterial dissection(s), and 17% of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection. Conclusions-In this registry, FMD occurred primarily in middle-aged women, although it presents across the lifespan. Cerebrovascular FMD occurred as frequently as renal FMD. Although a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.

Original languageEnglish (US)
Pages (from-to)3182-3190
Number of pages9
JournalCirculation
Volume125
Issue number25
DOIs
StatePublished - Jun 26 2012

Fingerprint

Fibromuscular Dysplasia
Registries
Aneurysm
Blood Vessels
Dissection
Stroke
Hypertension
Vertebral Artery
Tinnitus
Transient Ischemic Attack
Renal Artery
Sudden Death
Carotid Arteries
Headache
Pathologic Constriction
Epidemiology
Arteries
Kidney

Keywords

  • aneurysm
  • dissection
  • fibromuscular dysplasia
  • hypertension
  • renal
  • stroke

ASJC Scopus subject areas

  • Physiology (medical)
  • Cardiology and Cardiovascular Medicine

Cite this

Olin, J. W., Froehlich, J., Gu, X., Michael Bacharach, J., Eagle, K., Gray, B. H., ... Gornik, H. L. (2012). The United States registry for fibromuscular dysplasia: Results in the first 447 patients. Circulation, 125(25), 3182-3190. https://doi.org/10.1161/CIRCULATIONAHA.112.091223

The United States registry for fibromuscular dysplasia : Results in the first 447 patients. / Olin, Jeffrey W.; Froehlich, James; Gu, Xiaokui; Michael Bacharach, J.; Eagle, Kim; Gray, Bruce H.; Jaff, Michael R.; Kim, Esther S H; MacE, Pam; Matsumoto, Alan H.; McBane, Robert D.; Kline-Rogers, Eva; White, Christopher J.; Gornik, Heather L.

In: Circulation, Vol. 125, No. 25, 26.06.2012, p. 3182-3190.

Research output: Contribution to journalArticle

Olin, JW, Froehlich, J, Gu, X, Michael Bacharach, J, Eagle, K, Gray, BH, Jaff, MR, Kim, ESH, MacE, P, Matsumoto, AH, McBane, RD, Kline-Rogers, E, White, CJ & Gornik, HL 2012, 'The United States registry for fibromuscular dysplasia: Results in the first 447 patients', Circulation, vol. 125, no. 25, pp. 3182-3190. https://doi.org/10.1161/CIRCULATIONAHA.112.091223
Olin JW, Froehlich J, Gu X, Michael Bacharach J, Eagle K, Gray BH et al. The United States registry for fibromuscular dysplasia: Results in the first 447 patients. Circulation. 2012 Jun 26;125(25):3182-3190. https://doi.org/10.1161/CIRCULATIONAHA.112.091223
Olin, Jeffrey W. ; Froehlich, James ; Gu, Xiaokui ; Michael Bacharach, J. ; Eagle, Kim ; Gray, Bruce H. ; Jaff, Michael R. ; Kim, Esther S H ; MacE, Pam ; Matsumoto, Alan H. ; McBane, Robert D. ; Kline-Rogers, Eva ; White, Christopher J. ; Gornik, Heather L. / The United States registry for fibromuscular dysplasia : Results in the first 447 patients. In: Circulation. 2012 ; Vol. 125, No. 25. pp. 3182-3190.
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abstract = "Background-Fibromuscular dysplasia (FMD), a noninflammatory disease of medium-size arteries, may lead to stenosis, occlusion, dissection, and/or aneurysm. There has been little progress in understanding the epidemiology, pathogenesis, and outcomes since its first description in 1938. Methods and Results-Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from 9 US sites. Vascular beds were imaged selectively based on clinical presentation and local practice. The majority of patients were female (91{\%}) with a mean age at diagnosis of 51.9 (SD 13.4 years; range, 5-83 years). Hypertension, headache, and pulsatile tinnitus were the most common presenting symptoms of the disease. Self-reported family history of stroke (53.5{\%}), aneurysm (23.5{\%}), and sudden death (19.8{\%}) were common, but FMD in first-or second-degree relatives was reported only in 7.3{\%}. FMD was identified in the renal artery in 294 patients, extracranial carotid arteries in 251 patients, and vertebral arteries in 82 patients. A past or presenting history of vascular events were common: 19.2{\%} of patients had a transient ischemic attack or stroke, 19.7{\%} had experienced arterial dissection(s), and 17{\%} of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection. Conclusions-In this registry, FMD occurred primarily in middle-aged women, although it presents across the lifespan. Cerebrovascular FMD occurred as frequently as renal FMD. Although a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.",
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