A Prospective Study of the Renal Artery Fibromuscular Dysplasia Registry in China
Objective: 1) To describe the characteristics of renal artery FMD; 2) To identify environmental/ hormonal factors and exposures (for example smoking) associated with the onset and progression of renal artery FMD; 3)To identify baseline characteristics of the disease associated with an increased risk of complications such as dissections, aneurysms, stroke or myocardial infarction; 4) To provide evidence-based algorithms for the management and follow-up of patients with renal artery FMD; 5) To establish a comprehensive imaging resource including a wide range of presentations of renal artery FMD. 4. Study design: Prospective, multi-center, observational study. 5. Study population: renal artery fibromuscular dysplasia 6. Data Collections: 1) Data on demographic characteristics, clinical characteristics, blood routine, biochemical and plasmic electrolytes and vascular imaging were collected using a questionnaire; 2) The diagnosis of renal artery FMD was based on the identification of focal or multifocal FMD lesions in at least one arterial bed by computed tomography angiography, magnetic resonance angiography and/or digital subtraction angiographies; 3) For the patients with renal artery FMD, screening was performed to assess most arteries and multivessel FMD would be collected; 4) All patients would be followed up. 7. Treatment: Standardized diagnosis and treatment procedure as recommended in the International Consensus on the diagnosis and management of fibromuscular dysplasia. 8. Follow up: 3, 6, 12 months after diagnosis and every year after enrolled. 9. Sample size estimation: About 5 hundred. 10. Timeline: Start of subjects' enrollment: Jan 2021; End of subjects enrollment: December 2026; End of study: December 2036. 11. Organization: The Centre for Epidemiological Studies and Clinical Trials, Ruijin Hospital, Shanghai, China.
• Informed consent;
• Patients diagnosed with renal artery fibromuscular dysplasia (FMD), i.e. at least one string-of-beads (multifocal FMD) or focal stenosis (focal FMD). The diagnosis needs to be confirmed by at least one of the following imaging modalities: CT-angiography, MR-angiography or catheter-based angiography;
• Patients with so-called atypical renal artery FMD or renal artery FMD-like presentation, i.e. patients presenting with at least one dissection or 2 aneurysms \< 60-year-old, in the absence string-of-beads, focal stenosis or evidence of inherited arteriopathy;
• Patients with established renal artery FMD younger than 18 years, without evidence of underlying syndromes such as Williams syndrome, Alagille syndrome or type 1 Neurofibromatosis;