title: The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients.
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.
  
  
  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.
authors.affiliation: Mount Sinai School of Medicine, One Gustave L. Levy Place, Box 1033, New York, NY 10029, USA. jeffrey.olin@mountsinai.org
authors.forename: Jeffrey W
authors.initials: JW
authors.lastname: Olin
authors.forename: James
authors.initials: J
authors.lastname: Froehlich
authors.forename: Xiaokui
authors.initials: X
authors.lastname: Gu
authors.forename: J Michael
authors.initials: JM
authors.lastname: Bacharach
authors.forename: Kim
authors.initials: K
authors.lastname: Eagle
authors.forename: Bruce H
authors.initials: BH
authors.lastname: Gray
authors.forename: Michael R
authors.initials: MR
authors.lastname: Jaff
authors.forename: Esther S H
authors.initials: ES
authors.lastname: Kim
authors.forename: Pam
authors.initials: P
authors.lastname: Mace
authors.forename: Alan H
authors.initials: AH
authors.lastname: Matsumoto
authors.forename: Robert D
authors.initials: RD
authors.lastname: McBane
authors.forename: Eva
authors.initials: E
authors.lastname: Kline-Rogers
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: White
authors.forename: Heather L
authors.initials: HL
authors.lastname: Gornik
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D005352:Fibromuscular Dysplasia / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D012189:Retrospective Studies; D014481:United States / Q000453:epidemiology; D055815:Young Adult