Jeffrey Olin, DO, presented the Charles J. Tegtmeyer Annual Lecture at ISET 2019. Dr. Olin spoke about fibromuscular dysplasia (FMD) and described advances in the field as well as misconceptions about the disease.
“You might think this is a rare disease,” he said, “but it’s not. It’s rarely diagnosed, and there’s a long delay from first symptom to diagnosis.” He cited data from the CORAL trial, which was supposed to exclude patients with FMD and yet later showed that 9% of women included in the trial had FMD.
Dr. Olin described multifocal (“string of beads”) and focal FMD, which accounts for around 5% to 10% of cases. Knowledge surrounding the disease has advanced rapidly, he noted, and he showed audience members how a table from a 2004 New England Journal of Medicine article he had published is now known to be incorrect.
To learn more about FMD, Dr. Olin in 2008 started the United States Registry for FMD with funding from the FMD Society of America Foundation. “We now have over 2,400 patients in the registry,” said Dr. Olin. Registry data have revealed that patients with FMD have a mean age of 52 years rather than 20 to 30 years as was previously believed.
The presenting symptoms of FMD are fairly nonspecific and include hypertension, pulsatile tinnitus, headache, and dizziness. “You have to ask the patient about the pulsatile tinnitus. Do you hear a swooshing or a whooshing sound in your ear? They often don’t volunteer the information,” said Dr. Olin.
Dr. Olin said that 44% of the patients in their series had a dissection or aneurysm, and this prevalence has led his group to recommend that all patients with FMD have one-time, cross-sectional imaging from head to pelvis whether or not they have symptoms.
Additional recommendations include that all patients who have had a spontaneous coronary artery dissection (SCAD) should be on beta-blockers for the rest of their life, and “their blood pressure should be meticulously controlled,” said Dr. Olin.
SCAD, said Dr. Olin, is the primary manifestation of coronary artery FMD. “Arterial tortuosity may also be seen. The string of beads is not seen, and therefore stress testing, cardiac catheterization are not recommended to screen for [coronary artery FMD] because you will see normal arteries, other than tortuosity, prior to the SCAD event,” explained Dr. Olin.
Dr. Olin also described common misconceptions about FMD. These include:
- That looking at the angiogram will reveal how severely an artery is narrowed
- That visual inspection of the angiogram will show the effectiveness of the angiogram post intervention
- That because stents are the “norm” for atherosclerotic renal artery disease, they should be used for FMD.
He concluded his presentation with a list of recommendations outlining when to consider a diagnosis of FMD. FMD should be in the differential when the following characteristics are present:
- Onset of hypertension under age 35 years
- Difficult to control hypertension
- Epigastric bruit and high blood pressure
- Cervical bruit in anyone under age 60 years
- Pulsatile tinnitus
- Severe and recurrent headaches similar to a migraine
- TIA or stroke in a patient under age 60 years
- Dissection of a peripheral or coronary artery
- Aneurysm in a young patient
- Intracranial aneurysm
- Subarachnoid hemorrhage
- Renal infarction
Dr. Olin concluded by thanking the members of his team and the patient foundation for FMD. He also informed the audience that a forthcoming paper on the proteomics of FMD will describe a blood test that will determine whether someone has FMD.