Giant cell arteritis
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
Look out for this icon: for treatment options that are affected, or added, as a result of your patient's comorbidities.
suspected GCA
prednisone
Should be initiated immediately if index of suspicion for GCA is high. Treatment should not be delayed if results of acute-phase reactants (C-reactive protein/erythrocyte sedimentation rate) or temporal artery biopsy are pending.
Glucocorticoids have broad immunosuppressive and anti-inflammatory properties; the goal is to improve symptoms and prevent vision loss.
The dosing and duration of oral glucocorticoid therapy can be variable depending on a patient's manifestations and comorbidities and whether the use of a corticosteroid-sparing agent was also initiated.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com The dose of prednisone can be tapered (over several months) once remission has been achieved.
Relapses on reducing the dose are common.
Frequent dose adjustments may be required over time depending on response to therapy.
Primary options
prednisone: 1 mg/kg/day orally for 4 weeks, maximum 80 mg/day; gradually taper dose over several months once remission has been achieved
These drug options and doses relate to a patient with no comorbidities.
Primary options
prednisone: 1 mg/kg/day orally for 4 weeks, maximum 80 mg/day; gradually taper dose over several months once remission has been achieved
Drug choice, dose and interactions may be affected by the patient's comorbidities. Check your local drug formulary.
Show drug information for a patient with no comorbidities
Primary options
prednisone
methylprednisolone pulse therapy
Some newly diagnosed patients with threatened visual loss may benefit from an intravenous pulse-dose corticosteroid, but there is a risk of toxicity and the decision should be guided by the patient's clinical condition, values, and preferences.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com This should be followed with standard oral prednisone regimen.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com [63]Fraser JA, Weyand CM, Newman NJ, et al. The treatment of giant cell arteritis. Rev Neurol Dis. 2008 Summer;5(3):140-52. http://www.ncbi.nlm.nih.gov/pubmed/18838954?tool=bestpractice.com [66]Chan CC, Paine M, O'Day J. Steroid management in giant cell arteritis. Br J Ophthalmol. 2001 Sep;85(9):1061-4. http://www.ncbi.nlm.nih.gov/pubmed/11520757?tool=bestpractice.com
Primary options
methylprednisolone: 500-1000 mg intravenously once daily for 3-5 days
These drug options and doses relate to a patient with no comorbidities.
Primary options
methylprednisolone: 500-1000 mg intravenously once daily for 3-5 days
Drug choice, dose and interactions may be affected by the patient's comorbidities. Check your local drug formulary.
Show drug information for a patient with no comorbidities
Primary options
methylprednisolone
confirmed GCA
prednisone
Glucocorticoids have broad immunosuppressive and anti-inflammatory properties; the goal is to improve symptoms and prevent vision loss.
The dosing and duration of oral glucocorticoid therapy can be variable depending on a patient's manifestations and comorbidities and whether the use of a corticosteroid-sparing agent was also initiated.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com The dose of prednisone can be tapered (over several months) once remission has been achieved. When prednisone is used with tocilizumab, prednisone is typically tapered over 26 weeks.[67]Stone JH, Tuckwell K, Dimonaco S, et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med. 2017 Jul 27;377(4):317-28. http://www.ncbi.nlm.nih.gov/pubmed/28745999?tool=bestpractice.com
Relapses on reducing the dose are common.
Frequent dose adjustments may be required over time depending on response to therapy.
Primary options
prednisone: 1 mg/kg/day orally for 4 weeks, maximum 80 mg/day; gradually taper dose over several months once remission has been achieved
tocilizumab or methotrexate or abatacept
Treatment recommended for SOME patients in selected patient group
Tocilizumab, in combination with an accelerated prednisone taper, can be considered for use in patients with newly diagnosed disease who are at a particularly high risk for serious glucocorticoid-associated adverse events.[74]Antonio AA, Santos RN, Abariga SA. Tocilizumab for giant cell arteritis. Cochrane Database Syst Rev. 2022 May 13;5(5):CD013484. https://www.cochranelibrary.com/cdsr/doi/10.1002/14651858.CD013484.pub3/full http://www.ncbi.nlm.nih.gov/pubmed/35560150?tool=bestpractice.com The American College of Rheumatology/Vasculitis Foundation guideline has issued a conditional recommendation for the use of of oral glucocorticoids with tocilizumab over oral glucocorticoids alone.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com
Tocilizumab should also be considered for patients with refractory or relapsing GCA who require the ongoing use of moderate to high doses of glucocorticoids. Optimal usage of tocilizumab in routine clinical practice and its optimal duration remains to be determined. Lack of long-term follow-up data on tocilizumab and significant cost may limit its use.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com
Serious hepatotoxicity (including acute liver failure, hepatitis and jaundice) has been reported with tocilizumab. Use caution in patients with hepatic impairment. Monitor alanine aminotransferase (ALT) or aspartate aminotransferase (AST) before initiation of treatment, every 4 to 8 weeks for the first 6 months of treatment, and then every 12 weeks thereafter.[77]Medicines and Healthcare products Regulatory Agency. Tocilizumab (RoActemra): rare risk of serious liver injury including cases requiring transplantation. Jul 2019 [internet publication]. https://www.gov.uk/drug-safety-update/tocilizumab-roactemra-rare-risk-of-serious-liver-injury-including-cases-requiring-transplantation Other adverse events related to tocilizumab include serious infections and neutropenia, an alteration in lipid profiles, and bowel perforation.[78]Unizony S, McCulley TJ, Spiera R, et al. Clinical outcomes of patients with giant cell arteritis treated with tocilizumab in real-world clinical practice: decreased incidence of new visual manifestations. Arthritis Res Ther. 2021 Jan 6;23(1):8. https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-020-02377-8 http://www.ncbi.nlm.nih.gov/pubmed/33407817?tool=bestpractice.com [79]Campbell L, Chen C, Bhagat SS, et al. Risk of adverse events including serious infections in rheumatoid arthritis patients treated with tocilizumab: a systematic literature review and meta-analysis of randomized controlled trials. Rheumatology (Oxford). 2011 Mar;50(3):552-62. https://academic.oup.com/rheumatology/article/50/3/552/1790102 http://www.ncbi.nlm.nih.gov/pubmed/21078627?tool=bestpractice.com [80]Xie F, Yun H, Bernatsky S, et al. Brief report: risk of gastrointestinal perforation among rheumatoid arthritis patients receiving tofacitinib, tocilizumab, or other biologic treatments. Arthritis Rheumatol. 2016 Nov;68(11):2612-7. https://onlinelibrary.wiley.com/doi/epdf/10.1002/art.39761 http://www.ncbi.nlm.nih.gov/pubmed/27213279?tool=bestpractice.com [81]Strangfeld A, Richter A, Siegmund B, et al. Risk for lower intestinal perforations in patients with rheumatoid arthritis treated with tocilizumab in comparison to treatment with other biologic or conventional synthetic DMARDs. Ann Rheum Dis. 2017 Mar;76(3):504-10. https://ard.bmj.com/content/76/3/504.long http://www.ncbi.nlm.nih.gov/pubmed/27405509?tool=bestpractice.com
Randomized controlled trials evaluating the efficacy of methotrexate in patients with GCA have yielded conflicting results.[82]Hoffman GS, Cid MC, Hellmann DB, et al. A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum. 2002 May;46(5):1309-18. http://www.ncbi.nlm.nih.gov/pubmed/12115238?tool=bestpractice.com [83]Jover JA, Hernandez-Garcia C, Morado IC, et al. Combined treatment of giant-cell arteritis with methotrexate and prednisone: a randomized, double-blind, placebo-controlled trial. Ann Intern Med. 2001 Jan 16;134(2):106-14. http://www.ncbi.nlm.nih.gov/pubmed/11177313?tool=bestpractice.com However, evidence from one meta-analysis of individual patient data suggested that methotrexate may be effective at lowering the risk of first and second relapse, and exposure to glucocorticoids.[84]Mahr AD, Jover JA, Spiera RF, et al. Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum. 2007 Aug;56(8):2789-97. http://www.ncbi.nlm.nih.gov/pubmed/17665429?tool=bestpractice.com Methotrexate may be an alternative to tocilizumab in patients unable to use tocilizumab (e.g., due to recurrent infections, history of gastrointestinal perforations, or diverticulitis).[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com
In patients for whom tocilizumab and methotrexate are not effective or tolerated, abatacept may be useful when used with glucocorticoids.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com However, this recommendation is supported by one small randomized double-blind trial of 49 patients, in whom the addition of abatacept to a standardized prednisone taper was shown to be of benefit.[85]Langford CA, Cuthbertson D, Ytterberg SR, et al. A randomized, double-blind trial of abatacept (CTLA-4Ig) for the treatment of giant cell arteritis. Arthritis Rheumatol. 2017 Apr;69(4):837-45. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5378642 http://www.ncbi.nlm.nih.gov/pubmed/28133925?tool=bestpractice.com Specifically, the relapse-free survival rate at 12 months was 48% for those receiving abatacept and 31% for those receiving placebo (P=0.049).
Primary options
tocilizumab: 162 mg subcutaneously once weekly (a dose of 162 mg every 2 weeks may be prescribed based on clinical considerations); 6 mg/kg intravenously every 4 weeks, maximum 600 mg/dose
Secondary options
abatacept: body weight <60 kg: 500 mg intravenously every 2 weeks at week 0, 2, and 4, followed by 500 mg every 4 weeks starting at week 8; body weight 60-100 kg: 750 mg intravenously every 2 weeks at week 0, 2, and 4, followed by 750 mg every 4 weeks starting at week 8; body weight >100 kg: 1000 mg intravenously every 2 weeks at week 0, 2, and 4, followed by 1000 mg every 4 weeks starting at week 8
OR
methotrexate: 7.5 mg orally once weekly initially, dose may be increased slowly to a maximum of 20 mg once weekly; dose should be given on the same day of each week
aspirin
Treatment recommended for SOME patients in selected patient group
Retrospective studies suggest that patients with GCA receiving aspirin may have a lower risk of vision loss and stroke without increased risk of bleeding complications; however, this benefit is not universally acknowledged.[86]Nesher G, Berkun Y, Mates M, et al. Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis Rheum. 2004 Apr;50(4):1332-7. http://www.ncbi.nlm.nih.gov/pubmed/15077317?tool=bestpractice.com [87]Lee MS, Smith SD, Galor A, et al. Antiplatelet and anticoagulant therapy in patients with giant cell arteritis. Arthritis Rheum. 2006 Oct;54(10):3306-9. http://www.ncbi.nlm.nih.gov/pubmed/17009265?tool=bestpractice.com However, other observational studies have failed to replicate these findings.[88]Narváez J, Bernad B, Gómez-Vaquero C, et al. Impact of antiplatelet therapy in the development of severe ischemic complications and in the outcome of patients with giant cell arteritis. Clin Exp Rheumatol. 2008 May-Jun;26(3 suppl 49):S57-62. http://www.ncbi.nlm.nih.gov/pubmed/18799055?tool=bestpractice.com [89]Salvarani C, Della Bella C, Cimino L, et al. Risk factors for severe cranial ischaemic events in an Italian population-based cohort of patients with giant cell arteritis. Rheumatology (Oxford). 2009 Mar;48(3):250-3. https://www.doi.org/10.1093/rheumatology/ken465 http://www.ncbi.nlm.nih.gov/pubmed/19109317?tool=bestpractice.com One meta-analysis of observational studies reported a marginal benefit when antiplatelet/anticoagulant therapy was used together with corticosteroid therapy in patients with established GCA, without an associated increase in bleeding risk.[90]Martínez-Taboada VM, López-Hoyos M, Narvaez J, et al. Effect of antiplatelet/anticoagulant therapy on severe ischemic complications in patients with giant cell arteritis: a cumulative meta-analysis. Autoimmun Rev. 2014 Aug;13(8):788-94. http://www.ncbi.nlm.nih.gov/pubmed/24667078?tool=bestpractice.com
Low-dose aspirin treatment should be considered on an individual basis. It may benefit patients with GCA who have critical or flow-limiting involvement of the vertebral or carotid arteries.[34]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. 2021 Aug;73(8):1349-65. https://onlinelibrary.wiley.com/doi/10.1002/art.41774 http://www.ncbi.nlm.nih.gov/pubmed/34235884?tool=bestpractice.com
Primary options
aspirin: 100 mg orally once daily
osteoporosis prevention
Treatment recommended for SOME patients in selected patient group
Measures include preventing glucocorticoid-induced bone loss with optimized intake of dietary and supplemental calcium and vitamin D based on age-appropriate national recommended dietary allowances.[68]American College of Rheumatology. 2022 American College of Rheumatology guideline for the prevention and treatment of glucocorticoid-induced osteoporosis. Jul 2023 [internet publication]. https://rheumatology.org/glucocorticoid-induced-osteoporosis-guideline Patients ages ≥40 years receiving long-term glucocorticoids and deemed to be at high risk of fracture should receive an oral bisphosphonate. Other agents including intravenous bisphosphonates, parathyroid hormone/parathyroid hormone analogs (e.g., teriparatide, abaloparatide), or denosumab are also options. Selection of an agent should be based on patient and physician preferences.[68]American College of Rheumatology. 2022 American College of Rheumatology guideline for the prevention and treatment of glucocorticoid-induced osteoporosis. Jul 2023 [internet publication]. https://rheumatology.org/glucocorticoid-induced-osteoporosis-guideline Fracture assessment (including bone mineral density testing) is recommended within 6 months of starting glucocorticoid therapy for adults and every 1-2 years thereafter while continuing glucocorticoid therapy.[68]American College of Rheumatology. 2022 American College of Rheumatology guideline for the prevention and treatment of glucocorticoid-induced osteoporosis. Jul 2023 [internet publication]. https://rheumatology.org/glucocorticoid-induced-osteoporosis-guideline
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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
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