ATLANTA – One of the goals in soon-to-be-published Takayasu’s arteritis guidelines from the American College of Rheumatology is to wean patients off high-dose steroids once they are in remission.
This recommendation is in opposition to another option – namely, switching these patients to low-dose glucocorticoids. The idea is to prevent long-term side effects, particularly in children. The guidelines also recommend against escalating immunotherapy for asymptomatic increases in inflammatory markers and generally recommend against surgery – stenting in most cases – unless there is threat to life, limb, or organ and also if limb pain is so severe it cramps quality of life and dose escalation doesn’t get the job done. If surgery is planned, patients should be on perioperative steroids if there’s active disease.
It’s draft guidance for now, but it’s probably what the final document will say when it’s published in 2020, according to a presentation at the annual meeting of the American College of Rheumatology by one of the authors,, an associate professor of rheumatology at Northwestern University, Chicago. She gave a sneak preview at the meeting.
In general, severe, active Takayasu’s calls for high-dose oral steroids in conjunction with a nonsteroid immunosuppressive, such as methotrexate, azathioprine, leflunomide, or mycophenolate mofetil. There’s evidence that dual therapy gives a more durable remission and also reduces the need for steroids.
When that approach doesn’t do the trick, the next step is a tumor necrosis factor (TNF) inhibitor. There’s evidence for infliximab, adalimumab, certolizumab, and etanercept. Dr. Dua noted, “We still can consider” tocilizumab, but it failed to meet its primary endpoint in a randomized trial, and evidence for other biologics is sparse or nonexistent. “TNF inhibitors are the first line” for refractory disease, Dr. Dua said.
The steroid taper comes after 6-12 months of remission. Given their toxicity, “our goal for steroids is zero,” especially in pediatric populations. Even in remission, patients should have a clinical assessment, including inflammatory markers, every 3-12 months.
A rise in C-reactive protein or erythrocyte sedimentation rate, with no new symptoms, might be a reason for more frequent monitoring, but it’s not a reason to escalate immunosuppression. That should be kept in reserve for new vascular lesions, rapid progression on an old one, or worsening of organ or limb ischemia.
“We recommend [escalation] over surgical intervention” because patients often develop collateral circulation that solves the problem; it also gives the disease time to quiet down should the patient eventually go into surgery. Immediate surgery is reserved for organ or life-threatening disease, Dr. Dua said.
“Takayasu’s is different from other vasculitides in the sense that patients often present with certain nonspecific constitutional symptoms,” and there’s not a lot of pathology or histology to work with, “so we do tend to rely on imaging a lot,” Dr. Dua said.
Angiography has fallen out of favor because it’s invasive and exposes patients to radiation, among other problems. The field has moved to noninvasive imaging such as color Doppler ultrasound, CT angiography, magnetic resonance angiography, and PET CT.
“We do recommend regularly scheduled, noninvasive imaging every 6-12 months, in addition to the routine clinical assessment,” except in children with inactive disease; the risk of sedation outweighs the imaging benefit, Dr. Dua said.
In patients with single-vessel cranial or cervical stenosis, without symptoms, “we recommend medical over surgical management because of the risk of surgery. Surgery can be considered for multivessel involvement,” she said.
She and her colleagues also recommend medical management for renal artery stenosis, including antihypertensives and immunotherapy escalation for active disease. Surgery is considered for refractory hypertension or worsening kidney function
Dr. Dua is a primary investigator and adviser for Chemocentryx and an adviser for Novartis and AbbVie.