Giant Cell Arteritis and Takayasu Arteritis
Publication Date: July 8, 2021
Last Updated: January 4, 2023
Key Points and Definitions
Key Points and Definitions
- Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are systemic vasculitides that primarily affect large and medium-sized vessels.
- GCA can present with both cranial and extracranial manifestations and is more common in individuals of Northern European descent who are older than 50 years of age.
- TAK is more common in younger women and causes granulomatous inflammation of the aorta and its branches.
Table 1. Definitions of Selected Terms Used in the Recommendations and Ungraded Position Statements for GCA and TAK
| Term | Definition |
|---|---|
| Disease States | |
| Suspected disease | Clinical symptoms or signs suggestive of GCA/TAK and not explained by other conditions |
| Active disease | New, persistent, or worsening clinical signs and/or symptoms attributed to GCA/TAK and not related to prior damage |
| Severe disease | Vasculitis with life-/organ-threatening manifestations (e.g., vision loss, cerebrovascular ischemia, cardiac ischemia, limb ischemia) |
| Non-severe disease | Vasculitis without life-/organ-threatening manifestations (e.g., constitutional symptoms, headache, jaw claudication, symptoms of polymyalgia rheumatica) |
| Remission | Absence of clinical signs or symptoms attributed to active GCA/TAK, on or off of immunosuppressive therapy |
| Refractory disease | Persistent active disease despite an appropriate course of immunosuppressive therapy |
| Relapse | Recurrence of active disease following a period of remission |
| Cranial ischemia | Visual and neurological involvement including amaurosis fugax, vision loss, and stroke |
| Disease Assessments | |
| Clinical monitoring | Assessing for clinical signs and symptoms of active disease, obtaining 4 extremity blood pressures, and obtaining clinical labs including inflammatory markers |
| Inflammatory markers | Erythrocyte sedimentation rate, C-reactive protein |
| Non-invasive imaging | Computed tomography angiogram, magnetic resonance angiogram, positron emission tomography scan, vascular ultrasound, magnetic resonance imaging of temporal and scalp arteries |
| Invasive imaging | Conventional catheter-based angiogram |
Table 3. Treatments and Interventions Used in the Recommendations and Ungraded Position Statements for GCA and TAK
Table 3. Treatments and Interventions Used in the Recommendations and Ungraded Position Statements for GCA and TAK
| Term | Definition |
|---|---|
| Pulse intravenous glucocorticoids | Methylprednisolone:
|
| High dose oral glucocorticoids | Prednisone:
|
| Moderate dose oral glucocorticoids | Prednisone:
|
| Low dose oral glucocorticoids | Prednisone: ≤10 mg daily or equivalent |
| Non-glucocorticoid non-biologic immunosuppressive therapy | Azathioprine, leflunomide, methotrexate, mycophenolate mofetil, cyclophosphamide |
| Biologics | Abatacept, tumor necrosis factor (TNF) -α inhibitors, tocilizumab |
| Surgical intervention | Angioplasty, stent placement, vascular bypass, vascular graft |
Diagnostic Testing
Diagnostic Testing
Table 4. Recommendations for Diagnostic Testing in GCA
| Recommendation | Level of Evidence |
|---|---|
| In patients with suspected GCA, we conditionally recommend an initial unilateral temporal artery biopsy over bilateral biopsies. | Low |
| In patients with suspected GCA, we conditionally recommend a long-segment temporal artery biopsy (>1 cm) over a short-segment temporal artery biopsy (<1 cm). | Low |
| In patients with suspected GCA, we conditionally recommend obtaining a temporal artery biopsy specimen within two weeks of starting oral glucocorticoids over waiting longer than two weeks for a biopsy. | Low |
| In patients with suspected GCA, we conditionally recommend temporal artery biopsy over temporal artery ultrasound for diagnosis of GCA. | Low |
| In patients with suspected GCA, we conditionally recommend temporal artery biopsy over magnetic resonance imaging of the cranial arteries for establishing a diagnosis of GCA. | Low |
| In patients with suspected GCA and a negative temporal artery biopsy (or biopsies), we conditionally recommend non-invasive vascular imaging of the large vessels with clinical assessment to aid in diagnosis over clinical assessment alone. | Very low / Low |
| In patients with newly diagnosed GCA, we conditionally recommend obtaining non-invasive vascular imaging to evaluate for large vessel involvement. | Very low |
Treatment / Management of GCA
Treatment / Management of GCA
Table 4. Recommendations/Statements for Treatment (Medical and Surgical Management) and Clinical/Laboratory Monitoring in GCA
| A. Medical Management | Level of Evidence |
|---|---|
| In patients with newly diagnosed GCA without manifestations of cranial ischemia, we conditionally recommend initiating treatment with high dose oral glucocorticoids over pulse intravenous glucocorticoids. | Very low / Low |
| In patients with newly diagnosed GCA with threatened vision loss, we conditionally recommend initiating treatment with pulse intravenous glucocorticoids over high dose oral glucocorticoids. | Very low |
| In patients with newly diagnosed GCA, we conditionally recommend dosing oral glucocorticoids daily over an alternate day schedule. | Low |
| In patients with newly diagnosed GCA, we conditionally recommend initiating treatment with high dose oral glucocorticoids over moderate dose oral glucocorticoids. | Very low / Low |
| In patients with newly diagnosed GCA, we conditionally recommend using oral glucocorticoids with tocilizumab over oral glucocorticoids alone. | Low / High |
| In patients with GCA with active extracranial large vessel involvement, we conditionally recommend treatment with oral glucocorticoids combined with a non-glucocorticoid immunosuppressive agent over oral glucocorticoids alone. | Very low / Low |
| 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. | Low / Moderate |
| In patients with newly diagnosed GCA, we conditionally recommend against using an HMG-CoA reductase inhibitor (“statin”) specifically for the treatment of GCA. | Very low |
| In patients with GCA who have critical or flow-limiting involvement of the vertebral or carotid arteries, we conditionally recommend adding aspirin. | Very low / Moderate |
| In patients with GCA who experience disease relapse while on moderate or high dose glucocorticoids, we conditionally recommend adding a non-glucocorticoid immunosuppressive drug | Expert opinion |
| In patients with GCA who relapse with symptoms of cranial ischemia, we conditionally recommend adding a non-glucocorticoid immunosuppressive agent and increasing the dose of glucocorticoids over increasing the dose of glucocorticoids alone. | Expert opinion |
| In patients with GCA who experience disease relapse with symptoms of cranial ischemia while on glucocorticoids, we conditionally recommend adding tocilizumab and increasing the dose of glucocorticoids over adding methotrexate and increasing the dose of glucocorticoids. | Expert opinion |
| B. Surgical Management | Level of Evidence |
|---|---|
| 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. | N/A |
| In patients with severe GCA and worsening signs of limb/organ ischemia on immunosuppression, we conditionally recommend escalating immunosuppression over surgical intervention with escalation of immunosuppression. | Very low / Low |
| In patients with GCA undergoing vascular surgical intervention, we conditionally recommend using high dose glucocorticoids during the peri-procedural period if the patient has active disease. | Very low |
| C. Clinical/Laboratory Monitoring | Level of Evidence |
|---|---|
| In patients with GCA in apparent remission, we strongly recommend long-term clinical monitoring over no clinical monitoring. | Very low / Low |
| In patients with GCA who have rising inflammatory markers alone, we conditionally recommend clinical observation and monitoring without escalation of immunosuppression. | Very low |
Figure 1. Key Recommendations for the Treatment of GCA
Treatment / Management of TAK
Treatment / Management of TAK
Table 5. Recommendations/Statements for Medical and Surgical Management in TAK
| A. Medical Management | Level of Evidence |
|---|---|
| In patients with active, severe TAK not on immunosuppression, we conditionally recommend initiating treatment with high dose oral glucocorticoids over pulse intravenous glucocorticoids followed by high dose oral glucocorticoids | Very low |
| In patients with newly diagnosed active, severe TAK, we conditionally recommend initiating treatment with high-dose glucocorticoids over low-dose glucocorticoids. | Very low / Low |
| In patients with TAK who achieved remission on glucocorticoids for at least 6–12 months, we conditionally recommend tapering off glucocorticoids over long-term treatment with low dose glucocorticoids for remission maintenance. | Very low |
| In patients with active TAK, we conditionally recommend using a non-glucocorticoid immunosuppressive agent plus glucocorticoids over glucocorticoids alone. | Low |
| In patients with active TAK, we conditionally recommend using other non-glucocorticoid immunosuppressive therapy over tocilizumab as initial therapy. | Very low / Low |
| In patients with TAK refractory to treatment with glucocorticoids alone, we conditionally recommend adding a tumor necrosis factor inhibitor over adding tocilizumab. | Very low |
| In patients with TAK refractory to treatment with glucocorticoids alone, we conditionally recommend adding a tumor necrosis factor inhibitor over adding tocilizumab. | Very low |
| In patients with active TAK and critical cranial or vertebrobasilar involvement, we conditionally recommend adding aspirin or another anti-platelet therapy. | Low |
| B. Surgical Management | Level of Evidence |
|---|---|
| Ungraded Position Statement: For any patient requiring surgical vascular intervention, the type and timing of invention should be a collaborative decision between the vascular surgeon and rheumatologist. | N/A |
| In patients with known TAK and persistent limb claudication without evidence of ongoing active disease, we conditionally recommend against surgical intervention. | Very low / Low |
| In patients with known TAK with worsening signs of limb/organ ischemia on immunosuppression, we conditionally recommend escalating immunosuppression over surgical intervention with escalation of immunosuppression. | Very low |
| In patients with TAK with renovascular hypertension and renal artery stenosis, we conditionally recommend medical management over surgical intervention. | Very low / Low |
| In patients with TAK and stenosis of a cranial/cervical vessel without clinical symptoms, we conditionally recommend medical management over surgical intervention | Very low / Low |
| In 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. | Very low / Low |
| In patients with TAK undergoing surgical intervention, we conditionally recommend using high dose glucocorticoids in the peri-procedure period if the patient has active disease. | Very low / Low |
Table 6. Recommendations for Clinical/Laboratory Monitoring and Vascular Imaging in TAK
| A. Clinical/Laboratory Monitoring | Level of Evidence |
|---|---|
| In patients with TAK, we conditionally recommend adding inflammatory markers to clinical monitoring as a disease activity assessment tool. | Very low / Low |
| In patients with TAK in apparent remission, we strongly recommend long-term clinical monitoring over no clinical monitoring. | Very low |
| In patients with TAK in apparent clinical remission but with rising inflammatory markers, we conditionally recommend clinical observation without escalation of immunosuppression. | Very low |
| B. Vascular Imaging | Level of Evidence |
|---|---|
| In patients with TAK, we conditionally recommend using non-invasive imaging over catheter-based dye angiography as a disease activity assessment tool. | Low |
| In patients with known TAK, we conditionally recommend regularly scheduled non-invasive imaging in addition to routine clinical assessment | Very low / Low |
| In 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. | Very low / Low |
Figure 2. Overview of Treatment of TAK Based on Clinical and Radiographic Assessments
Recommendation Grading
Source Citation
Maz M, Chung SA, Abril A, Langford CA, Gorelik M, Guyatt G, Archer AM, Conn DL, Full KA, Grayson PC, Ibarra MF, Imundo LF, Kim S, Merkel PA, Rhee RL, Seo P, Stone JH, Sule S, Sundel RP, Vitobaldi OI, Warner A, Byram K, Dua AB, Husainat N, James KE, Kalot MA, Lin YC, Springer JM, Turgunbaev M, Villa-Forte A, Turner AS, Mustafa RA. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Rheumatol. 2021 Aug;73(8):1349-1365. doi: 10.1002/art.41774. Epub 2021 Jul 8. PMID: 34235884.
Disclaimer
This pocket guide attempts to define principles of practice that should produce high-quality patient care. It is applicable to specialists, primary care, and providers at all levels. This pocket guide should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. The ultimate judgment concerning the propriety of any course of conduct must be made by the clinician after consideration of each individual patient situation. Neither IGC, the medical associations, nor the authors endorse any product or service associated with the distributor of this clinical reference tool.