ACR/Vasculitis Foundation Guideline for Giant Cell Arteritis and Takayasu Arteritis Save

The ACR and the Vasculitis Foundation have published their evidence-based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK).

Guidelines were developed by a voting panel of using GRADE methodology and answering a series of PICO questions GCA and TAK (27 for GCA, 27 for TAK). Systematic literature reviews were conducted for each PICO question, based on a systematic literature review and expert consensus. Recommendation required ≥70% consensus among the Voting Panel. The Voting Panel included 9 adult rheumatologists, 5 pediatric rheumatologists, and 2 patients.

These recommendations address a variety of clinical questions and scenarios including diagnostic testing, imaging, treatments, and surgical interventions in GCA and TAK. Recommendations for GCA and TAK include support for the use of glucocorticoid-sparing immunosuppressive agents and the use of imaging to identify large vessel involvement. Recommendations for TAK include the use of glucocorticoids as initial therapy. There were only 2 strong recommendations; the remaining recommendations were conditional (expert opinion) due to the low quality of evidence available for most PICO questions.

In the end, the panel put forth 22 recommendations and 2 ungraded position statements for GCA; and 20 recommendations and 1 ungraded position statements for TAK. Ungraded position statements reflect the lack of evidence for one intervention over the other, but since these are commonly encountered clinical questions, they labeled such guidance as “ungraded position statements". These are noted in italics below.

Recommendations for the management of GCA

• Diagnostic testing
  1. Recommendation: For patients with suspected GCA, we conditionally recommend an initial unilateral temporal artery biopsy over bilateral biopsies.
  2. Recommendation: For patients with suspected GCA, we conditionally recommend a long-segment temporal artery biopsy specimen (>1 cm) over a short-segment temporal artery biopsy specimen (<1 cm).
  3. Recommendation: For patients with suspected GCA, we conditionally recommend obtaining a temporal artery biopsy specimen within 2 weeks of starting oral glucocorticoids over waiting longer than 2 weeks for a biopsy. 
  4. Recommendation: For patients with suspected GCA, we conditionally recommend temporal artery biopsy over temporal artery ultrasound for establishing a diagnosis of GCA. 
  5. Recommendation: For patients with suspected GCA, we conditionally recommend temporal artery biopsy over magnetic resonance imaging (MRI) of the cranial arteries for establishing a diagnosis of GCA.
  6. Recommendation: For patients with suspected GCA and a negative temporal artery biopsy result (or results), we conditionally recommend noninvasive vascular imaging of the large vessels with clinical assessment to aid in diagnosis over clinical assessment alone.
  7. Recommendation: For patients with newly diagnosed GCA, we conditionally recommend obtaining noninvasive vascular imaging to evaluate large vessel involvement.
• Medical management
  1. Recommendation: For patients with newly diagnosed GCA without manifestations of cranial ischemia, we conditionally recommend initiating treatment with high-dose oral glucocorticoids over intravenous (IV) pulse glucocorticoids.
  2. Recommendation: For patients with newly diagnosed GCA with threatened vision loss, we conditionally recommend initiating treatment with IV pulse glucocorticoids over high-dose oral glucocorticoids.
  3. Recommendation: For patients with newly diagnosed GCA, we conditionally recommend dosing oral glucocorticoids daily over an alternate-day schedule.
  4. Recommendation: For patients with newly diagnosed GCA, we conditionally recommend initiating treatment with high-dose oral glucocorticoids over moderate-dose oral glucocorticoids.
  5. Recommendation: For patients with newly diagnosed GCA, we conditionally recommend the use of oral glucocorticoids with tocilizumab over oral glucocorticoids alone.
  6. Recommendation: For patients with GCA with active extracranial large vessel involvement, we conditionally recommend treatment with oral glucocorticoids combined with a nonglucocorticoid immunosuppressive agent over oral glucocorticoids alone.
  7. Ungraded position statement: The optimal duration of therapy with glucocorticoids for GCA is not well established and should be guided by the patient’s values and preferences.
  8. Recommendation: In patients with newly diagnosed GCA, we conditionally recommend against the use of a hydroxymethylglutaryl-coenzyme A reductase inhibitor (“statin”) specifically for the treatment of GCA.
  9. Recommendation: For patients with GCA who have critical or flow-limiting involvement of the vertebral or carotid arteries, we conditionally recommend adding aspirin.
  10. Recommendation: For patients with GCA who experience disease relapse while receiving moderate-to-high–dose glucocorticoids, we conditionally recommend adding a nonglucocorticoid immunosuppressive drug.
  11. Recommendation: For patients with GCA who experience disease relapse with symptoms of cranial ischemia, we conditionally recommend adding a nonglucocorticoid immunosuppressive agent and increasing the dose of glucocorticoids over increasing the dose of glucocorticoids alone.
  12. Recommendation: For patients with GCA who experience disease relapse with cranial symptoms while receiving glucocorticoids, we conditionally recommend adding tocilizumab and increasing the dose of glucocorticoids over adding methotrexate and increasing the dose of glucocorticoids.
• Surgical intervention
  1. Ungraded position statement: For any patient requiring surgical vascular intervention for GCA, the type and timing of intervention should be a collaborative decision between the vascular surgeon and rheumatologist.
  2. Recommendation: For patients with severe GCA and worsening signs of limb/organ ischemia who are receiving immunosuppressive therapy, we conditionally recommend escalating immunosuppressive therapy over surgical intervention with escalation of immunosuppressive therapy.
  3. Recommendation: For patients with GCA undergoing vascular surgical intervention, we conditionally recommend the use of high-dose glucocorticoids during the periprocedural period, if the patient has active disease.
• Clinical/laboratory monitoring
  1. Recommendation: For patients with GCA in apparent clinical remission, we strongly recommend long-term clinical monitoring over no clinical monitoring.
  2. Recommendation: For patients with GCA who have an increase in levels of inflammation markers alone, we conditionally recommend clinical observation and monitoring without escalation of immunosuppressive therapy.

Recommendations for the management of TAK

• Medical management
  1. Recommendation: For patients with active, severe TAK who are not receiving immunosuppressive therapy, we conditionally recommend initiating treatment with high-dose oral glucocorticoids over IV pulse glucocorticoids followed by high-dose oral glucocorticoids.
  2. Recommendation: For patients with newly active, severe TAK, we conditionally recommend initiating treatment with high-dose glucocorticoids over low-dose glucocorticoids.
  3. Recommendation: For patients with TAK who achieved remission while receiving glucocorticoids for ≥6–12 months, we conditionally recommend tapering off glucocorticoids over long-term treatment with low-dose glucocorticoids for remission maintenance.
  4. Recommendation: For patients with active TAK, we conditionally recommend the use of a nonglucocorticoid immunosuppressive agent plus glucocorticoids over glucocorticoids alone.
  5. Recommendation: For patients with active TAK, we conditionally recommend the use of other nonglucocorticoid immunosuppressive therapy over tocilizumab as initial therapy.
  6. Recommendation: For patients with TAK that is refractory to treatment with glucocorticoids alone, we conditionally recommend adding a tumor necrosis factor inhibitor over adding tocilizumab.
  7. Recommendation: For patients with TAK and asymptomatic progression of a previously identified vascular lesion seen on imaging, without evidence of inflammation, we conditionally recommend continuing current therapy over escalating/changing immunosuppressive therapy.
  8. Recommendation: For patients with active TAK and critical cranial or vertebrobasilar involvement, we conditionally recommend adding aspirin or another antiplatelet therapy.
• Clinical/laboratory monitoring
  1. Recommendation: For patients with TAK, we conditionally recommend adding inflammation markers to clinical monitoring as a disease activity assessment tool.
  2. Recommendation: For patients with TAK in apparent clinical remission, we strongly recommend long-term clinical monitoring over no clinical monitoring.
  3. Recommendation: For patients with TAK in apparent clinical remission but with an increase in levels of inflammation markers, we conditionally recommend clinical observation without escalation of immunosuppressive therapy.
  4. Recommendation: For patients with TAK, we conditionally recommend the use of noninvasive imaging over catheter-based dye angiography as a disease activity assessment tool.
  5. Recommendation: For patients with known TAK, we conditionally recommend regularly scheduled noninvasive imaging in addition to routine clinical assessment.
  6. Recommendation: For patients with TAK in apparent clinical remission but with signs of inflammation in new vascular territories (e.g., new stenosis or vessel wall thickening) on vascular imaging, we conditionally recommend treatment with immunosuppressive therapy.
• Surgical intervention
  1. Ungraded position statement: For any patient requiring surgical vascular intervention, the type and timing of intervention should be a collaborative decision between the vascular surgeon and rheumatologist.
  2. Recommendation: In patients with known TAK and persistent limb claudication without evidence of ongoing active disease, we conditionally recommend against surgical intervention.
  3. Recommendation: For patients with known TAK with worsening signs of limb/organ ischemia while receiving immunosuppressive therapy, we conditionally recommend escalating immunosuppressive therapy over surgical intervention with escalation of immunosuppressive therapy.
  4. Recommendation: For patients with TAK with renovascular hypertension and renal artery stenosis, we conditionally recommend medical management over surgical intervention.
  5. Recommendation: For patients with TAK and stenosis of a cranial/cervical vessel without clinical symptoms, we conditionally recommend medical management over surgical intervention.
  6. Recommendation: For patients with TAK with worsening signs of limb/organ ischemia, we conditionally recommend delaying surgical intervention until the disease is quiescent over performing surgical intervention while the patient has active disease.
  7. Recommendation: For patients with TAK who are undergoing surgical intervention, we conditionally recommend the use of high-dose glucocorticoids in the periprocedure period if the patient has active disease.