The ACR recently developed a draft of its first-ever evidence-based guidelines for the diagnosis, treatment, and management of patients with systemic vasculitis. Monday morning, three people involved in the process used a case-based approach to demonstrate how to apply the new recommendations for giant cell arteritis (GCA), Takayasu’s arteritis (TAK), and polyarteritis nodosa (PAN).
The new guidelines include both “strong” recommendations, which are generally supported by moderate or high-quality evidence, and “conditional” recommendations, which are generally supported by lower-quality evidence. Session moderator Sharon Chung, MD, MAS, Director of the Vasculitis Clinic at the University of California, San Francisco, said given the lack of high-quality evidence, the majority of the recommendations are conditional. She said she expects the final publication of the recommendations to happen in 2020.
Giant Cell Arteritis
Mehrdad Maz, MD, Professor of Medicine and Rheumatology Fellowship Program Director at the University of Kansas Medical Center, Kansas City, began by illustrating how to use the new recommendations for GCA with the case of a 71-year-old Caucasian female, taking attendees through the recommendations regarding diagnosis, treatment, and monitoring.
Dr. Maz reviewed the status of the case two-and-a-half years following diagnosis to illustrate the one strong recommendation for GCA.
“She has not had any recurrence of cranial or extracranial symptoms off immunosuppressive therapies,” he said. “Her labs are satisfactory, including inflammatory markers, and her exam is unrevealing. Does the patient need long-term monitoring or could she follow up with her primary care physician and return to the rheumatology clinic as needed?”
According to the guidelines, in patients with GCA in apparent remission, long-term clinical monitoring is strongly recommended.
Other highlights from the GCA guideline includes recommending daily glucocorticoid dosing rather than alternate-day dosing and using tocilizumab to sustain remission while reducing glucocorticoid use.
As with GCA, the strong recommendation for TAK relates to clinical monitoring. Anisha Dua, MD, MPH, Associate Professor and Rheumatology Fellowship Program Director at Northwestern University, Chicago, said the TAK guideline strongly recommend long-term clinical monitoring of patients with TAK every three to 12 months.
“A consideration to keep in mind, is how long they’ve been in remission. So, obviously, early on in the disease you might be monitoring them more frequently,” she said. “You also have to consider the site of involvement, risks for disease progression, what baseline immunosuppressive therapy they received, and perhaps most importantly, their reliability in recognizing and reporting the symptoms of this disease. A lot of patients may under-report things because they worry your first response will be to prescribe steroids, so you have to ask.”
Clinical monitoring should be supplemented with lab and imaging evaluations.
For patients in remission, Dr. Dua said, another key take-home point is the goal to try to completely wean them off steroids. The guideline also recommends monitoring for increases in ESR and CRP — as long as there are no new symptoms — without escalating immunosuppressive therapy.
Jason Springer, MD, MS, Director of the Vasculitis Center of Excellence at the University of Kansas Medical Center, described the guideline for PAN, including a look at recommendations for adenosine deaminase 2 deficiency, or DADA2.
“In patients with PAN and DADA2, we strongly recommend using TNF-α inhibitors, such as infliximab, etanercept, or adalimumab, over glucocorticoids alone,” Dr. Springer said. “There’s convincing evidence that has shown TNF-α inhibitors can prevent some of the critical and catastrophic vascular incidents, such as stroke. Physicians should be clued in to DADA2 when a patient has PAN-like symptoms and stroke or TIA.”
Dr. Springer also looked at a case of patient with PAN with disease refractory to treatment with glucocorticoids alone. The guidelines conditionally recommend adding cyclophosphamide over increasing the glucocorticoid dose alone, he said.