While vasculitis remains rare, giant cell arteritis (GCA) is an urgent priority for primary care as the population ages. Experts warn that with vision loss occurring in 20% of untreated cases, early detection is vital.
Primary care clinicians serve as the critical first line of defense against the most common form of vasculitis in older adults, according to experts at the American College of Physicians Internal Medicine (ACP-IM) Meeting 2026 in San Francisco.
A shift in treatment, marked by the 2025 approval of the first oral targeted therapy, moves management back to frontline clinicians, who must now balance the promise of steroid-free remission against new, serious cardiovascular and infection risks, said Ora Singer, MD, MS, associate professor of internal medicine in the Division of Rheumatology at the University of Michigan, Ann Arbor, Michigan, during a presentation on management of vasculitis.
Clinical manifestations of the condition include headaches, jaw pain, fatigue, and scalp tenderness. Patients may also present with symptoms of polymyalgia rheumatica, which include pain and stiffness in the shoulders and hip girdle. Visible beading of the superficial temporal artery is a hallmark sign, Singer said. An estimated 10 cases per 100,000 occur in people older than age 50, but rates are higher among those of Scandinavian ancestry.
Early identification is important to prevent serious complications from GCA such as vision loss, blood clots, and aneurysms, said Arundathi Jayatilleke, MD, rheumatologist and professor of medicine at the Lewis Katz School of Medicine at Temple University in Philadelphia, who was not involved with the presentation.
Up to 20% of patients with the condition experience vision loss, which can become permanent if untreated.
A workup of potential GCA should rule out malignancy, infection, or stroke, Singer said. Around 5% of patients with the condition have normal erythrocyte sedimentation rate and C-reactive protein results on laboratory testing, she said.
Imaging may show characteristic vascular inflammation, especially in the temporal, axillary, and carotid arteries, and a distinctive dark halo sign on ultrasound. A positive temporal artery biopsy is definitive for diagnosis, but a negative test does not rule out disease.
GCA usually presents with an initial flare, which is followed by treatment and remission, Jayatilleke said.
“These patients are usually best served with initial close management by a rheumatologist, followed by monitoring in primary care, depending on the course of their illness,” she said.
Patients with the condition are immunosuppressed, so primary care clinicians should monitor for infection and ensure that patients are current with recommended vaccines, Singer said.
Medication Evolution
Patients diagnosed with GCA should start treatment with glucocorticoids immediately to avoid vision loss, Singer said. Doses vary depending on the presence or absence of eye involvement. Previously, the standard of care was to taper the medication over a year.
Long-term treatment of the condition with corticosteroids calls for monitoring of a range of clinical factors including blood pressure, glycemic control, bone density, and gastrointestinal concerns.
But the discovery and approval of two newer agents have led to quicker tapers and a significant reduction in steroid exposure for this population, Singer explained.
Tocilizumab, an interleukin-6 receptor alpha inhibitor, was approved for the treatment of GCA in 2017 after demonstrating higher rates of sustained glucocorticoid-free remission compared to placebo. However, tocilizumab is delivered as a weekly injection or monthly infusion, presenting a barrier for some patients.
“This drug reduces the acute-phase reactants; thus, the Westergren sedimentation rate and C-reactive protein cannot be used to monitor disease activity in patients on tocilizumab,” Singer said.
Upadacitinib, an oral JAK inhibitor, is the only oral targeted therapy for GCA, approved in 2025. In a phase 3 trial, upadacitinib was superior to placebo for sustaining glucocorticoid-free disease remission after 1 year.
Upadacitinib comes with a black box warning for increased risk for major cardiovascular events such as myocardial infarction and cerebrovascular accident. Use of the drug has also been associated with a higher risk for herpes zoster, so patients should receive the shingles vaccine, ideally prior to starting the drug, Singer said.
Singer disclosed no financial conflicts of interest. Jayatilleke disclosed no financial conflicts of interest.
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