For some patients, it starts with a headache. Or jaw pain. Or scalp tenderness. If a discerning physician thinks that these symptoms could indicate something more sinister, they will want their patient to get an immediate diagnosis to confirm or rule out giant cell arteritis (GCA), a condition that, if left untreated, can cause blindness.
For Paula Hooper, it started with a sore throat that affected only the left side of her neck. When a headache set in and it hurt to keep her eyes open, Hooper knew that something strange and serious was afoot. She emailed her Brigham rheumatologist, Jeffrey Sparks, MD, MMSc, and then went to go shower.
“On my way from the kitchen to the bathroom, he called,” Hooper remembered. “He told me, ‘You need to get to the Brigham. Right now.’”
On the car ride to the hospital, Hooper noticed that the vision in her left eye was beginning to blur.
To save a patient’s vision, two key factors are needed: speed and accuracy. That’s what led the Brigham to launch its Fast Track Clinic for Giant Cell Arteritis. The clinic features a multidisciplinary team of specialists with expertise in the diagnosis and treatment of GCA, including rheumatologists, vascular medicine specialists, vascular and endovascular surgeons, and pathologists who work together to rapidly evaluate and care for patients.
“Early diagnosis is essential,” said William Docken, MD, co-director of the Fast Track Clinic for GCA and medical director of the Brigham’s Orthopaedics and Arthritis Center at Chestnut Hill. “There is a lot of skill involved in diagnosing or ruling out GCA, which means that doing it accurately requires extensive experience. At the Brigham, we have both the technology and the expertise to skillfully diagnose and manage GCA. There are few places like ours in the country and in the world.”
Helping Patients Like Paula
GCA is an inflammatory disease that only affects adults older than age 50. Its wide-ranging symptoms are caused by inflammation in the lining of the arteries, most often those in the head, and especially around the temples. Once diagnosed, GCA needs to be treated as quickly as possible with high-dose steroids, usually for two weeks, then slowly tapered. If a patient hasn’t yet lost their vision, this treatment will usually protect them from vision loss. But high-dose steroids can cause serious side effects, so it’s important to neither over nor undertreat the condition.
For Hooper, her diagnosis came just in time. After she was seen at the Fast Track Clinic for GCA, Sparks sent her to the Brigham’s Emergency Department where she received intravenous steroids.
“I remember how quickly everyone moved once I got to the hospital,” said Hooper. “But they were also so sweet — especially my ultrasound tech.”
Magda Abdou, RPVI, RVT, RPhS, RDMS, senior vascular technologist and clinical education coordinator for the Brigham’s Vascular Diagnostic Lab, is the technologist Hooper remembers. Abdou is also one of the driving forces behind the Fast Track Clinic’s record of success. A former physician from Egypt, Abdou researched and advocated for a way of diagnosing GCA that, before the Fast Track Clinic’s launch, was only popular in Europe.
“Magda brought with her a wealth of knowledge. She’s the kind of person who does the research, tracks the data and follows the patients,” said her colleague Deborah Hand, RVT, chief vascular sonographer.
Rapid Evaluation, Advanced Treatment
The Brigham is among the few national hospitals that use noninvasive vascular ultrasound in the diagnosis of GCA. The technique involves performing an ultrasound examination of the temporal arteries, their branches and the large arteries of the upper chest and arms, allowing physicians to see signs of swelling of the artery wall caused by GCA. In active GCA, a dark, thickening of the blood vessel wall, known as a “halo sign,” can be seen.
In the U.S., temporal artery biopsy — in which a small sample of the artery is taken and evaluated — is the most common way to diagnose GCA. But both Docken and Abdou were intrigued by the promise of ultrasound and its success in Europe.
“Magda and I began talking, and the idea grew to start doing noninvasive vascular ultrasound here,” said Docken. “We now have a cadre of skilled ultrasound technologists who can do this test.”
Ultrasound for GCA remains uncommon in the U.S. At most centers that do perform it, rheumatologists both perform and evaluate ultrasound images. But the Brigham is unique; highly trained ultrasound technologists from the Vascular Diagnostic Laboratory team have performed the technique for more than six years. Images are then formally evaluated and interpreted a physician. The final reports are done by Piotr Sobieszczyk, MD, co-director of the Fast Track Clinic for GCA and a specialist in Vascular Medicine.
“The Fast Track Clinic for GCA represents both a collaboration at the physician level with clinicians from two different sub-specialties — cardiovascular and rheumatology — working closely together and learning from each other as well as a collaboration with the vascular lab technologists and staff,” said Sobieszczyk. “This program has been a source of satisfaction and pride, especially for the technologists. Some have had opportunities to travel to take courses to learn about new techniques and have brought those back to teach them to others. It’s been a positive experience for all of us.”
Building a Case for Ultrasound
Since 2015, the Brigham’s multidisciplinary team has performed more than 1,100 ultrasounds to diagnose or evaluate patients for GCA. According to Sara Tedeschi, MD, MPH, a physician in the Division of Rheumatology, this is the largest cohort in the U.S. Her team is evaluating how ultrasound is being used at the Brigham, including what symptoms lead to referrals and how test results have impacted diagnosis and treatment. She’s studying both patients who have been referred to the Fast Track Clinic as well as those who have already been diagnosed and are now being monitored.
“The use of ultrasound for evaluating GCA at the Brigham has become much more common since the test first became available in 2013,” said Tedeschi. “In that year, there were 40 to 50 ultrasounds ordered to evaluate for GCA. Now, it’s being widely ordered not just by rheumatologists but also by neurologists, cardiologists and primary medical teams treating hospitalized patients.”
Hooper is deeply grateful for Sparks’ referral and the care she received.
“With GCA, I was down for the count,” Hooper recalls. “But the Brigham is excellent. The doctors really listened to me and answered my questions. I get nervous about it coming back, but now I know the signs to watch for.”