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Background: Fibromuscular dysplasia (FMD), a nonatherosclerotic arterial disease, can cause pain and vascular complications. The aim of this study was to examine the impact of FMD symptoms and complications on quality of life, depression, anxiety, and self-rated health., Design: This was a cross-sectional, correlational study., Methods: Participants were adults with a diagnosis of FMD. Quality of life (36-Item Short Form Health Survey), anxiety and depression (Patient-Reported Outcomes Measurement Information System [PROMIS®]), self-rated health question, and symptom/complication questionnaires were mailed to patients with FMD. Scores were compared with symptoms and complications. Multivariable linear models were fit for symptoms and survey scores. Ordinal regression was used for self-rated health. Backwards selection was run for each model. Alpha of 0.05 and 95% confidence intervals were used., Results: Of the 162 (275 total; 47.8%) patients who returned surveys (156 female), 130 had carotid or vertebral artery involvement (80.2%). Migraine (p < .001), neck pain (p = .036), and flank pain (p = .025) were associated with decrease in Mental Component scores. Migraine (p = .002) and neck pain (p = .023) were associated with lower Physical Component scores. Patients reporting abdominal pain compared with those without had 4.88 points higher depression. Abdominal pain (p = .031) and pulsatile tinnitus (p = .011) were associated with greater anxiety. Migraine was associated with (p = .002) lower self-rated health. Participants with history of stroke/transient ischemic attack had 2.42 (1.08, 5.46; p = .033) times the odds of poor self-rated health compared with those without stroke/transient ischemic attack., Conclusions: Among patients with FMD, presence of pain and history of vascular complications were related to lower quality of life and self-rated health., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2021 Elsevier Ltd. All rights reserved.)

Importance: Fibromuscular dysplasia (FMD) is a nonatherosclerotic arterial disease that predominately affects women and is most commonly diagnosed in middle age. The natural history of FMD among patients diagnosed at an older age is not well understood., Objective: To examine the differences in clinical presentation, arterial bed involvement, vascular events, and need for vascular procedures between younger and older patients with FMD., Design, Setting, and Participants: Analysis of baseline data for patients enrolled in the US Registry for FMD as of December 15, 2016, at referral centers participating in the US Registry for FMD. Patients 18 years and older at the time of enrollment and those with only confirmed multifocal (string of beads type) FMD were included. Patients were categorized according to age at the time of diagnosis (≥65 years vs <65 years)., Main Outcomes and Measures: Prevalence of specific symptoms, vascular events, and prior vascular procedures at the time of enrollment in the registry., Results: A total of 1016 patients were included in the analysis, of whom, 170 (16.7%) were 65 years or older at the time of diagnosis. Older patients with FMD were more likely to be asymptomatic at the time of diagnosis (4.2% vs 1.4%; P = .02). Headache and pulsatile tinnitus, both common manifestations of FMD, were less common in older patients (40.5% vs 69.1%; P < .001 and 30% vs 44.6%; P < .001, respectively). Extracranial carotid arteries were more commonly involved in patients 65 years or older at time of diagnosis (87% vs 79.4%; P = .03). There was no difference in prevalence of renal artery involvement, number of arterial beds involved, or diagnosis of any aneurysm. Patients 65 years or older were less likely to have had a major vascular event (37.1% vs 46.1%; P = .03) and fewer had undergone a therapeutic vascular procedure (18.5% vs 33.1%; P < .001)., Conclusions and Relevance: In the US Registry for FMD, patients 65 years or older at the time of diagnosis of multifocal FMD were more likely to be asymptomatic, had lower prevalence of major vascular events, and had undergone fewer therapeutic vascular procedures than younger patients. Patients with multifocal FMD diagnosed at an older age may have a more benign phenotype and fewer symptoms.

The article presents a scientific statement from the American Heart Association about fibromuscular dysplasia (FMD), a nonatherosclerotic, noninflammatory vascular disease that may lead to arterial stenosis, occlusion, aneurysm, or dissection. Topics include an historical perspective on FMD, the prevalence of FMD, and genetic considerations. Histopathological classifications for FMD and clinical manifestations of FMD are also mentioned.

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. [ABSTRACT FROM AUTHOR]