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
prednisolone
If you strongly suspect GCA, start treatment with a high-dose glucocorticoid immediately while awaiting confirmation of the diagnosis.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
This means that you suspect GCA is a more likely diagnosis, based on the patient’s presentation, than any other condition, taking into account the symptoms, signs, and available laboratory test results.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Standard initial treatment for patients is oral prednisolone prescribed in a single daily dose.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com High-dose therapy is recommended to achieve rapid disease control. Actively consider other diagnoses while awaiting confirmation, and also if patients do not respond within 7 days.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Assess symptoms of and/or exposure to serious infections in all patients starting glucocorticoids, taking local prevalence of infections into account.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com Ask about exposure to tuberculosis and follow national screening guidelines.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com [60]National Institute for Health and Care Excellence. Tuberculosis. Feb 2024 [internet publication]. https://www.nice.org.uk/guidance/ng33
Ensure that patients are evaluated for hypertension and hyperglycaemia at the time of starting a high-dose glucocorticoid.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Primary options
prednisolone: 40-60 mg orally once daily initially, adjust dose according to response
More prednisoloneThere is no evidence-based regimen that can be used effectively, and dose regimens may vary. The dose presented here is taken from British Society for Rheumatology guidelines.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com Some experts recommend an initial dose of 1 mg/kg/day (maximum 60 mg/day) for 4 weeks. Continue at the same dose until symptoms and acute phase markers resolve.
These drug options and doses relate to a patient with no comorbidities.
Primary options
prednisolone: 40-60 mg orally once daily initially, adjust dose according to response
More prednisoloneThere is no evidence-based regimen that can be used effectively, and dose regimens may vary. The dose presented here is taken from British Society for Rheumatology guidelines.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com Some experts recommend an initial dose of 1 mg/kg/day (maximum 60 mg/day) for 4 weeks. Continue at the same dose until symptoms and acute phase markers resolve.
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
prednisolone
Consider – specialist treatment of visual or neurological symptoms
specialist treatment of visual or neurological symptoms
Additional treatment recommended for SOME patients in selected patient group
More aggressive treatment may be indicated for patients who present with recent vision loss.
In patients with GCA with acute or intermittent visual loss or amaurosis fugax, a specialist may consider giving intravenous methylprednisolone.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Referral for intravenous glucocorticoid therapy should not delay starting treatment with an oral glucocorticoid.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Vision loss due to anterior ischaemic optic neuropathy (ION) or central retinal artery obstruction from GCA is irreversible. Greater disease awareness and earlier diagnosis probably accounts for a decline in the incidence of permanent visual loss due to GCA.[43]Smith SCM, Al-Hashimi MR, Jones CD, et al. Frequency of visual involvement in a 10-year interdisciplinary cohort of patients with giant cell arteritis. Clin Med (Lond). 2023 May;23(3):206-12. https://www.doi.org/10.7861/clinmed.2022-0415 http://www.ncbi.nlm.nih.gov/pubmed/37197804?tool=bestpractice.com [61]Singh AG, Kermani TA, Crowson CS, et al. Visual manifestations in giant cell arteritis: trend over 5 decades in a population-based cohort. J Rheumatol. 2015 Feb;42(2):309-15. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4367485 http://www.ncbi.nlm.nih.gov/pubmed/25512481?tool=bestpractice.com
Primary options
methylprednisolone sodium succinate: 500-1000 mg intravenously once daily for up to 3 days, then switch to oral prednisolone
Secondary options
prednisolone: 60-100 mg orally once daily for up to 3 days, adjust dose according to response and taper gradually
These drug options and doses relate to a patient with no comorbidities.
Primary options
methylprednisolone sodium succinate: 500-1000 mg intravenously once daily for up to 3 days, then switch to oral prednisolone
Secondary options
prednisolone: 60-100 mg orally once daily for up to 3 days, adjust dose according to response and taper gradually
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 sodium succinate
Secondary options
prednisolone
confirmed GCA
prednisolone
Oral prednisolone is prescribed in a single daily dose to be administered in the morning after breakfast.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com High-dose therapy is recommended to achieve rapid disease control.
Assess symptoms of and/or exposure to serious infections in all patients starting glucocorticoids, taking local prevalence of infections into account.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com Ask about exposure to tuberculosis and follow national screening guidelines.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com [60]National Institute for Health and Care Excellence. Tuberculosis. Feb 2024 [internet publication]. https://www.nice.org.uk/guidance/ng33
Guidelines recommend tapering the dose over 12 to 18 months while monitoring for toxicity and relapse.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com Consider a more rapid dose reduction in patients at high risk of glucocorticoid toxicity and/or those receiving concomitant glucocorticoid-sparing therapy.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com However, there is a lack of good quality evidence on tapering regimens. In our expert’s opinion, most patients need 2 years of prednisolone treatment for GCA.[62]Mukhtyar C, Cate H, Graham C, et al. Development of an evidence-based regimen of prednisolone to treat giant cell arteritis - the Norwich regimen. Rheumatol Adv Pract. 2019 Feb 1;3(1):rkz001. https://www.doi.org/10.1093/rap/rkz001 http://www.ncbi.nlm.nih.gov/pubmed/31431989?tool=bestpractice.com
During prednisolone taper, evaluate the patient regularly by clinical examination and check inflammatory markers periodically. The treatment must be tapered in a safe manner with regular checks for toxicity and relapse. Diagnose a relapse objectively and not on the basis of just a rise in inflammatory markers. Reassess the patient as at baseline for other differential diagnoses and re-evaluate the glucocorticoid tapering plan.
Return of symptoms needs objective assessment for relapsing disease as at diagnosis. The temptation to treat a rise in C-reactive protein or headaches and jaw pain should be avoided. Intensification of immunosuppression must be justified by demonstration of relapsing disease whenever possible. Relapses require a change in the planned glucocorticoid therapy with addition of further immunosuppression in the form of methotrexate or tocilizumab (see below).
Ensure that patients are evaluated for hypertension and hyperglycaemia at the time of starting high-dose glucocorticoids.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Management of glucocorticoid-induced adverse events
Take measures to avoid possible complications associated with glucocorticoid treatment, such as bone protection with calcium, vitamin D, and bisphosphonates to prevent glucocorticoid-induced bone loss.[39]Mollan SP, Paemeleire K, Versijpt J, et al. European Headache Federation recommendations for neurologists managing giant cell arteritis. J Headache Pain. 2020 Mar 17;21(1):28. https://www.doi.org/10.1186/s10194-020-01093-7 http://www.ncbi.nlm.nih.gov/pubmed/32183689?tool=bestpractice.com [63]Grossman JM, Gordon R, Ranganath VK, et al. American College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Arthritis Care Res (Hoboken). 2010 Nov;62(11):1515-26. https://www.doi.org/10.1002/acr.20295 http://www.ncbi.nlm.nih.gov/pubmed/20662044?tool=bestpractice.com Counselling on lifestyle changes and monitoring may also be required.[63]Grossman JM, Gordon R, Ranganath VK, et al. American College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Arthritis Care Res (Hoboken). 2010 Nov;62(11):1515-26. https://www.doi.org/10.1002/acr.20295 http://www.ncbi.nlm.nih.gov/pubmed/20662044?tool=bestpractice.com Also consider providing a proton-pump inhibitor for gastrointestinal protection when prescribing high-dose glucocorticoid therapy. With lower glucocorticoid doses, coprescription of a proton-pump inhibitor may not be needed.[64]Scarpignato C, Gatta L, Zullo A, et al. Effective and safe proton pump inhibitor therapy in acid-related diseases - A position paper addressing benefits and potential harms of acid suppression. BMC Med. 2016 Nov 9;14(1):179. https://www.doi.org/10.1186/s12916-016-0718-z http://www.ncbi.nlm.nih.gov/pubmed/27825371?tool=bestpractice.com Follow your local guidance.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Primary options
prednisolone: 40-60 mg orally once daily initially, adjust dose according to response and taper gradually
More prednisoloneTapering regimens may vary; consult your local guidance. The British Society for Rheumatology guidelines recommends tapering the dose of prednisolone once clinical remission is achieved. The specific tapering regimen depends on the dose of prednisolone the patient is on.[36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
Consider – specialist treatment with tocilizumab or methotrexate
specialist treatment with tocilizumab or methotrexate
Additional treatment recommended for SOME patients in selected patient group
Tocilizumab or methotrexate may be considered by a specialist in combination with the glucocorticoid taper in patients with GCA who relapse, have refractory disease, or who are at high risk of glucocorticoid toxicity.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com
The interleukin-6 receptor inhibiting monoclonal antibody tocilizumab has been shown to be effective in patients with GCA, with one randomised controlled trial showing substantial reduction in the need for glucocorticoid therapy.[69]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 [70]Villiger PM, Adler S, Kuchen S, et al. Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet. 2016 May 7;387(10031):1921-7. http://www.ncbi.nlm.nih.gov/pubmed/26952547?tool=bestpractice.com [71]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
A Cochrane review concluded that tocilizumab is an effective and safe glucocorticoid‐sparing therapy in patients with newly diagnosed and relapsing GCA.[72]Antonio AA, Santos RN, Abariga SA. Tocilizumab for giant cell arteritis. Cochrane Database Syst Rev. 2022 May 13;(5):CD013484. https://www.doi.org/10.1002/14651858.CD013484.pub3 http://www.ncbi.nlm.nih.gov/pubmed/35560150?tool=bestpractice.com Adverse effects of tocilizumab include hepatic injury (see below), serious infections and neutropenia, an alteration in lipid profiles, and bowel perforation.[73]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://www.doi.org/10.1186/s13075-020-02377-8 http://www.ncbi.nlm.nih.gov/pubmed/33407817?tool=bestpractice.com [74]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://www.doi.org/10.1093/rheumatology/keq343 http://www.ncbi.nlm.nih.gov/pubmed/21078627?tool=bestpractice.com [75]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://www.doi.org/10.1002/art.39761 http://www.ncbi.nlm.nih.gov/pubmed/27213279?tool=bestpractice.com [76]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://www.doi.org/10.1136/annrheumdis-2016-209773 http://www.ncbi.nlm.nih.gov/pubmed/27405509?tool=bestpractice.com
Guidelines state that methotrexate may be used as an alternative to tocilizumab, for example in those unable to use tocilizumab due to recurrent infections, history of gastrointestinal perforations, or diverticulitis.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com [77]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, in the opinion of our expert, methotrexate may be considered first line as a glucocorticoid-sparing option because it has been in clinical use for longer than tocilizumab and is less toxic.
More information: Tocilizumab and methotrexate
One randomised trial found that patients with active GCA who received tocilizumab with glucocorticoid taper had higher rates of sustained remission at 52 weeks and fewer relapses than patients who received glucocorticoid taper alone.[71]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 Overall adverse events were similar, but serious adverse events were less frequent among those receiving tocilizumab.
In an open-label follow-up, a substantial proportion of patients who were in clinical remission following treatment with tocilizumab for 52 weeks maintained drug-free remission during the 2 years after tocilizumab cessation. Tocilizumab-based regimens restored clinical remission among patients who experienced relapse in the follow-up.[78]Stone JH, Han J, Aringer M, et al; for the GiACTA investigators. Long-term effect of tocilizumab in patients with giant cell arteritis: open-label extension phase of the Giant Cell Arteritis Actemra (GiACTA) trial. Lancet Rheumatol. 2021 May;3(5):e328-36. https://www.thelancet.com/journals/lanrhe/article/PIIS2665-9913(21)00038-2/fulltext Systematic reviews also suggest that tocilizumab reduces relapse rates and glucocorticoid requirements.[79]Monti S, Águeda AF, Luqmani RA, et al. Systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis: focus on giant cell arteritis. RMD Open. 2019 Sep 16;5(2):e001003. https://www.doi.org/10.1136/rmdopen-2019-001003 http://www.ncbi.nlm.nih.gov/pubmed/31673411?tool=bestpractice.com [80]Berti A, Cornec D, Medina Inojosa JR, et al. Treatments for giant cell arteritis: Meta-analysis and assessment of estimates reliability using the fragility index. Semin Arthritis Rheum. 2018 Aug;48(1):77-82. http://www.ncbi.nlm.nih.gov/pubmed/29496228?tool=bestpractice.com
Tocilizumab is given in combination with a tapering course of a glucocorticoid, but its optimal usage in routine clinical practice and its optimal duration remain to be determined, and it is associated with significant cost.
In England and Wales, the National Institute of Health and Care Excellence states that tocilizumab, when used with a tapering course of glucocorticoids (and when used alone after glucocorticoids), is recommended as an option for treating GCA in adults, only if:[81]National Institute for Health and Care Excellence. Tocilizumab for treating giant cell arteritis. April 2018 [internet publication]. https://www.nice.org.uk/guidance/ta518
They have relapsing or refractory disease
They have not already had tocilizumab
Tocilizumab is stopped after 1 year of uninterrupted treatment at most
The company provides it with the discount agreed in the patient access scheme.
Randomised 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
Drug safety information: Hepatic injury with tocilizumab
Cumulative analysis of safety data has identified cases of tocilizumab-related serious hepatic injury, including acute liver failure, hepatitis, and jaundice. Serious liver injury has been reported from 2 weeks to more than 5 years after starting treatment.
Alanine aminotransferase (ALT) or aspartate aminotransferase (AST) levels should be measured before starting treatment with tocilizumab and monitored every 4 to 8 weeks for the first 6 months of treatment followed by every 12 weeks thereafter. Specialists should exercise caution when considering use of tocilizumab in patients with hepatic impairment, particularly in patients with ALT or AST higher than 1.5 times the upper limit of normal. Initiation of treatment is not recommended in patients with ALT or AST higher than 5 times the upper limit of normal.[85]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
Primary options
tocilizumab: 162 mg subcutaneously once weekly
OR
methotrexate: 7.5 mg orally/subcutaneously once weekly (on the same day of each week) initially, increase gradually according to response, maximum 25 mg/week
More methotrexateSome experts may recommend a starting dose of 20 mg/week.
aspirin
Additional treatment recommended for SOME patients in selected patient group
Do not routinely give antiplatelet agents or anticoagulants.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com However, they may be considered on an i ndividual basis for select patients with vascular ischaemic complications or who are at high risk of cardiovascular disease.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com Follow national guidance on the secondary prevention of coronary and other atherosclerotic vascular diseases.
More information: Low-dose aspirin
Prevention of platelet aggregation with low-dose aspirin is potentially effective in preventing ischaemic complications of GCA. Retrospective chart reviews suggest that the risk of vision loss and stroke is lower, and the risk of bleeding complications was increased, in patients with GCA receiving aspirin.[35]Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020 Jan;79(1):19-30. https://www.doi.org/10.1136/annrheumdis-2019-215672 http://www.ncbi.nlm.nih.gov/pubmed/31270110?tool=bestpractice.com [36]Mackie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatology (Oxford). 2020 Mar 1;59(3):e1-e23. https://www.doi.org/10.1093/rheumatology/kez672 http://www.ncbi.nlm.nih.gov/pubmed/31970405?tool=bestpractice.com However, other observational studies have failed to replicate these findings.[86]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 [87]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 glucocorticoid therapy in patients with established GCA, without an associated increase in bleeding risk.[88]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
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aspirin: 75 mg orally once daily
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