Daniella Kadian-Dodov, MD | Fibromuscular Dysplasia: Looking Beyond the String-of-Beads

Scientific Concepts and Discoveries Presented

• Fibromuscular Dysplasia (FMD)
  • FMD is characterized by heterogeneous presentations affecting various arteries, primarily impacting women in their prime.
  • The condition is often identified through angiographic classification, distinguishing between multifocal (string of beads) and focal (single area of stenosis) forms.
  • Historical context: First identified in a young boy with hypertension, leading to further studies and classifications in the 1960s and 1970s.
• Pathophysiology
  • FMD is a non-atherosclerotic, non-inflammatory disease typically affecting mid to distal arteries.
  • The disease can lead to complications such as aneurysms and dissections, with a significant prevalence in renal and cervical arteries.
• Diagnosis and Classification
  • Diagnosis often involves imaging techniques like catheter-based angiography and CTA.
  • Important mimickers of FMD include vasospasm and dissection healing, which can present similarly on imaging.
• Epidemiology
  • The prevalence of FMD may be underestimated, with studies indicating it could affect millions of women in the U.S.
  • Recent findings suggest that FMD is not as rare as previously thought, with incidental findings in healthy kidney donors and patients undergoing imaging for unrelated reasons.
• Genetic and Environmental Factors
  • Genetic predispositions and environmental triggers (e.g., mechanical stress, smoking) may contribute to the development of FMD.
  • Familial cases and studies on identical twins indicate a potential genetic component, though the exact mechanisms remain unclear.
• Clinical Manifestations
  • Symptoms vary based on the artery involved, with hypertension being a common presenting sign for renal artery FMD.
  • A significant number of patients with FMD also have associated conditions such as spontaneous coronary artery dissection (SCAD) and aneurysms.
• Management and Follow-Up
  • Management strategies include lifestyle modifications and careful monitoring, particularly for those with aneurysms or dissections.
  • Recommendations against certain activities (e.g., intense isometric exercise, chiropractic neck manipulation) due to increased risk of dissection.
  • Regular follow-up imaging is advised, particularly for patients with identified vascular anomalies.

Methodology and Key Points

• Diagnosis Approach
  • Use of imaging (CTA preferred) for screening in patients with spontaneous dissections or aneurysms.
  • Regular follow-up and monitoring of patients with FMD, especially those with multifocal or focal lesions.
• Management Recommendations
  • Aspirin therapy (81 mg daily) for all patients with FMD.
  • Avoidance of specific activities that may increase dissection risk.
  • Genetic testing may be warranted in cases of multiple vascular findings.

Researchers and Sources Featured

• Daniella Kadian-Dodov, MD: Presenter and primary researcher discussing FMD.
• Dr. Olan: Senior partner and mentor to Dr. Kadian-Dodov, involved in FMD research.
• Dr. Kichic: Principal investigator for the Define FMD study.
• Annette King: Conducts biopsies for patient enrollment.
• Valentina: Laboratory technician managing patient samples.
• Dr. Olan's team: Various collaborators in the research efforts.