2021 ACR/Vasculitis Foundation Recommendations for the Management of Giant Cell Arteritis and Takayasu Arteritis

The American College of Rheumatology (ACR) and Vasculitis Foundation recently released guidelines for the clinical management of giant cell arteritis (GCA) and Takayasu arteritis (TAK). The full report was published in Arthritis & Rheumatology.

Because GCA and TAK have similar clinical profiles, guidelines regarding their management were developed concurrently. The population, intervention, comparator, and outcome (PICO) format was used to formulate clinical questions with regard to GCA and TAK. Systematic literature reviews were conducted to address each PICO question (27 each for GCA and TAK). The Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology was used to rate the quality of evidence identified in the literature. An in-person panel including 11 patients with vascular conditions reviewed the evidence report provided by the literature team. Finally, a voting panel of 9 adult rheumatologists, 5 pediatric rheumatologists, and 2 patients selected a series of recommendations. A recommendation required a voting panel consensus of 70% or more for publication. Recommendation strength was based on the quality of literature data.

Based on data from 399 articles, the ACR published 22 recommendations and 2 ungraded position statements for GCA regarding diagnostic testing, medical management, surgical intervention, and clinical/laboratory monitoring. In addition, from 347 articles, 20 recommendations and 1 ungraded position statement were provided for TAK regarding medical management, surgical intervention, clinical/laboratory monitoring, and vascular imaging. Due to the paucity of literature data, 2 recommendations were considered to be strong, while the remaining were conditional.

Recommendations for Diagnostic Testing of GCA

For patients with suspected GCA, the panelists recommend an initial unilateral temporal artery biopsy over bilateral biopsies.
For diagnostic purposes, obtaining a long-segment temporal artery biopsy specimen (>1 cm) has been recommended over a short-segment temporal artery biopsy specimen (<1 cm).
Temporal artery biopsy specimens should be collected within 2 weeks of initiation with oral glucocorticoids (GCs).
Temporal artery biopsy is recommended over temporal artery ultrasound and magnetic resonance imaging (MRI) of the cranial arteries for establishing a diagnosis of GCA.
For patients with suspected GCA and a negative temporal artery biopsy result, the panelists recommend noninvasive vascular imaging of the large vessels with clinical assessment compared with clinical assessment alone.
For patients with newly diagnosed GCA, large vessel involvement can be evaluated by noninvasive vascular imaging.

Recommendations for Medical Management of GCA

For patients with newly diagnosed GCA without manifestations of cranial ischemia, high-dose oral GCs are recommended over intravenous (IV) pulse GCs.
However, IV pulse GCs are recommended over high-dose oral GCs for patients at risk for vision loss.
Treatment for patients with newly-diagnosed GCA may be administered daily as opposed to an alternate-day schedule.
Initiating treatment with high-dose oral GCs may be preferred to moderate-dose oral GCs.
For patients with newly-diagnosed GCA, the panelists recommend concomitant use of tocilizumab with oral GCs over oral GCs alone.
For patients with GCA and active extracranial large vessel involvement, treatment with oral GCs in combination with a non-GC immunosuppressant agent has been recommended over oral GCs alone.
In patients with newly diagnosed GCA, the panelists recommend against the use of statins for treatment of GCA.
Aspirin has been recommended as an add-on treatment for patients with GCA with critical or flow-limiting involvement of the vertebral or carotid arteries.
For patients who experience disease relapse while receiving moderate- to high-dose GCs, the panelists recommend adding a non-GC immunosuppressive agent.
For patients who experience disease relapse with symptoms of cranial ischemia, the panelists recommend adding a non-GC immunosuppressive agent and increasing the dose of GCs over increasing the dose of GCs alone.
For patients who experience disease relapse with symptoms of cranial ischemia while receiving GCs, tocilizumab may be added and the dose of GCs be increased over adding methotrexate and increasing the dose of GCs.
In an ungraded position statement, the panelists noted that the optimal duration of GC therapy for GCA is unclear and should be “guided by the patient’s values and preferences.”

Recommendations for Surgical Intervention in GCA

For patients with severe GCA and worsening signs of limb/organ ischemia who are receiving immunosuppressive therapy, escalating immunosuppressive therapy has been recommended over surgical intervention with escalation of immunosuppressive therapy.
For patients with active GCA who undergo vascular surgical intervention, high-dose GCs have been recommended during the periprocedural period.

In an ungraded position statement, the panelists suggested that the circumstances of the surgery should be a “collaborative decision” between the vascular surgeon and the treating rheumatologist.

Recommendations for Clinical/Laboratory Monitoring of GCA

The panelists strongly recommend long-term clinical monitoring of GCA over no clinical monitoring in patients with GCA in clinical remission.
For patients with GCA who experience an increase in inflammation markers, clinical observation and monitoring may be performed without escalation of immunosuppressive therapy.

Recommendations for Medical Management of TAK

For patients with active, severe TAK who are not receiving immunosuppressive therapy, initiating treatment with high-dose oral GCs has been recommended over initiating treatment with IV pulse GCs followed by high-dose oral GCs.
Patients with newly active, severe TAK should receive initial treatment with high-dose GCs over low-dose GCs.
Patients in TAK remission following more than 6 to 12 months of treatment with GCs can be tapered off GCs. This has been recommended over long-term treatment with low-dose GCs for remission maintenance.
Patients with active TAK may receive treatment with a non-GC immunosuppressive agent plus GCs over GCs alone.
Other non-GC immunosuppressive therapy may be used over the tocilizumab as initial treatment.
For patients with TAK refractory to GCs, the addition of a tumor necrosis factor inhibitor (TNFi) has been recommended over the addition of tocilizumab.
For patients with TAK and asymptomatic progression of a vascular lesion, without evidence of inflammation, the panelists recommend continuing current therapy over escalating or changing current immunosuppressive therapy.
For patients with active TAK with critical cranial or vertebrobasilar involvement, the addition of aspirin or another antiplatelet therapy has been recommended.

Recommendations for Surgical Intervention in TAK

For patients with TAK and persistent limb claudication, without evidence of active disease, surgical intervention may not be recommended.
For patients with TAK and escalating signs of limb/organ ischemia, the panelists recommend escalation of immunosuppressive therapy over surgical intervention with escalation of immunosuppressive therapy.
For patients with TAK with renovascular hypertension and renal artery stenosis, medical management may be preferred over surgical intervention.
For patients with TAK and stenosis of a cranial/cervical vessel without clinical symptoms, medical management has been recommended over surgical intervention.
For patients with TAK and escalating signs of limb/organ ischemia, the panelists recommend delaying surgical intervention until the disease is quiescent over performing surgery while the disease is active.
High-dose GCs are recommended for the periprocedural period if the patient has active disease.
In an ungraded position statement, the panelists recommend that the circumstances of the surgery should be a “collaborative decision” between the surgeon and the treating rheumatologist.

Recommendations for Clinical/Laboratory Monitoring of TAK

Long-term clinical monitoring has been strongly recommended over no clinical/laboratory monitoring.
Inflammation markers may be added to clinical monitoring as a disease activity assessment tool.
Clinical observation without immunosuppressive treatment escalation may be recommended for patients in clinical remission who present with an increase in levels of inflammation markers.

Recommendations for Vascular Imaging in TAK

Noninvasive imaging may be preferred over catheter-based dye angiography as a disease activity assessment tool.
The panelists recommend regularly scheduled noninvasive imaging in addition to clinical monitoring.
Treatment with immunosuppressive therapy has been recommended for patients in clinical remission who present with signs of inflammation in vascular territories on imaging.

These recommendations address uncertainties regarding the diagnosis, treatment, monitoring, and management of GCA and TAK. However, gaps in the literature remain, and further research is necessary to develop stronger guidelines for the management of GCA and TAK.

“We are hopeful that additional investigations into GCA and TAK will enable a more tailored approach to disease management in order to improve outcomes and minimize treatment toxicities,” the authors of the guidelines wrote.

Disclosure: Several study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of disclosures.

Reference

Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of giant cell arteritis and Takayasu arteritis. Arthritis Rheumatol. Published online July 8, 2021. doi:10.1002/art.41774