Consensus score: 9.53. There is insufficient evidence to recommend any other oral immunosuppressive agent in GCA, including azathioprine, leflunomide or mycophenolate mofetil. FDG-PET can be useful for assessment of vascular inflammation, although it provides less detailed anatomic definition of the involved arteries compared with MRA or CTA. Conditional recommendation: The standard initial glucocorticoid dose for GCA is 40–60 mg oral prednis(ol)one per day. Part of the work is funded by Reuma Nederland. For relapse management, see Table 3. 7. Patients should be signposted to relevant patient support groups or charities as sources of peer support. Most occurrences of blindness or stroke happen either before treatment or during the first week of treatment . Dario Camellino – Travel expenses, consultancy and speaker fees from AbbVie, Celgene, Janssen-Cilag, Eli Lilly, Mylan and Sanofi. Most occurrences of blindness or stroke happen either before treatment or during the first week of treatment . The following evidence-based recommendations are graded as strong or conditional, with the quality of the evidence given as ++++ to + (unless no evidence was found) and a consensus score to indicate mean strength of agreement. 1,2 Initially GCA was considered a vasculitis affecting the carotid and vertebral artery branches only but was later redefined to include all medium and large vessels when autopsies showed involvement of large vessels in 80% of cases. With the approval of tocilizumab (TOC), which speci … NICE has accredited the process used by the BSR to produce its guideline on the diagnosis and treatment of giant cell arteritis. As new-onset headache is one of the principal symptoms of cranial GCA, neurologists often assess (and indeed may manage) people with this condition, in isolation from rheumatology. A 69-year-old white woman presented with a four-week history of severe pain in her neck, upper back and arms. Please check for further notifications by email. Giant cell arteritis (GCA) is a granulomatous vasculitis of large and medium-sized arteries. Consensus score: 9.61. UK prescribers should be aware that at the time of writing a limited duration of tocilizumab therapy for GCA has been approved by the Scottish Medicines Consortium and by National Health Service England for defined patient groups, taking into account cost-effectiveness data available at the time of the technology appraisal by the National Institute for Health and Care Excellence (TA518). Depending on the clinical situation, initiation of glucocorticoid treatment in primary care may be advised — the standard initial dose for GCA without visual symptoms is 40–60 mg oral prednisolone per day. Consensus score: 9.00. Symptoms of temporal arteritis. High dose glucocorticoid therapy (40–60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active giant cell arteritis (GCA) or Takayasu arteritis (TAK). 2. GCA typically occurs in people 50 years of age or older and is more common in women. Acute visual loss due to ocular ischaemia in GCA requires immediate action. Peter A. Merkel – Consulting fees from AbbVie, AstraZeneca, Biogen, Boeringher-Ingelheim, Bristol-Myers Squibb, Celgene, ChemoCentryx, CSL Behring, Genentech/Roche, Genzyme/Sanofi, GlaxoSmithKline, InflaRx, Insmed, Janssen and Kiniksa and research support from Bristol-Myers Squibb and Genentech/Roche/Chugai. In the absence of clinical features of cranial GCA, temporal artery biopsy can still be positive, but imaging of the extracranial large vessels may be considered instead of, or in addition to, temporal artery biopsy. QoE: +. As GCA is considered a medical emergency, it is treated at the point of diagnosis by clinicians in primary and secondary care who have a wide variety of clinical backgrounds. Note that for a medium (20–50%) estimated probability of GCA, it may be useful to perform an ultrasound prior to biopsy, in case the biopsy is negative. As well as confirmatory tests for GCA (see Key Recommendation 1), alternative explanations for patients’ symptoms should be considered, particularly if these confirmatory tests are negative. Consensus score: 9.61. Many patients with GCA have inflammation of the aorta and its proximal branches (extracranial large-v… Since involvement of the aorta and its proximal branches in GCA may be asymptomatic or associated only with constitutional symptoms, in some circumstances directed vascular imaging of the aorta and its proximal branches can be useful to detect inflammation, stenosis or dilatation. Temporal arteritis (giant cell arteritis) is where the arteries, particularly those at the side of the head (the temples), become inflamed. The doctor may recommend supplements such as calcium and vitamin D, and exercises such as walking or weight-bearing exercises, to prevent osteoporosis. [ 1 ] The first-line treatment for giant cell arteritis remains glucocorticosteroids. Giant cell arteritis is a serious disease that requires prompt medical attention since delay in diagnosis and treatment can lead to vision loss or other complications. Medication is continued at this high dose for approximately one month and then tapered gradually over the following months to the lowest possible dose, or is discontinued. Difficult-to-treat rheumatoid arthritis: contributing factors and burden of disease, A rare case of small-vessel necrotizing vasculitis of the bone marrow revealing granulomatosis with polyangiitis, Defining colchicine resistance/intolerance in patients with familial Mediterranean fever: a modified-Delphi consensus approach, Real-world single centre use of JAK inhibitors across the rheumatoid arthritis pathway, The management of Sjögren’s syndrome: British Society for Rheumatology guideline scope, About the British Society for Rheumatology, https://doi.org/10.1093/rheumatology/kez664, treatment This is the executive summary of British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis, doi: 10.1093/rheumatology/kez672, https://academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model, Receive exclusive offers and updates from Oxford Academic, British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis, Large vessel giant cell arteritis suggested by magnetic resonance imaging of the thigh: a potential mimicker of myositis, fasciitis and skeletal muscle vasculitis, Large-vessel involvement in recent-onset giant cell arteritis: a case–control colour-Doppler sonography study, Prognosis of large-vessel giant cell arteritis, Giant cell arteritis and polymyalgia rheumatica: usefulness of vascular magnetic resonance imaging studies in the diagnosis of aortitis. QoE: insufficient evidence. In all cases, taper schedules should be individualized based on the patient. The guideline also includes practical information for clinicians including what symptoms to check, what tests to do, steroid dosing and care pathways. Ultrasound can assess the axillary arteries, but ultrasound evaluation of the deeper arteries is more difficult. Participated in clinical trials sponsored by GlaxoSmithKline and Kiniksa. QoE: +++. Table 1 summarizes recommended assessments for patients with GCA. Conditional recommendation: Glucocorticoid dose should be tapered to zero over 12–18 months, providing there is no return of GCA symptoms, signs or laboratory markers of inflammation. Maria C. Cid – Research grant from Kiniksa and consulting fees from AbbVie and Janssen. Each general principle carries a consensus score (mean rating on a 0–10 scale). It furthers the University's objective of excellence in research, scholarship, and education by publishing worldwide, This PDF is available to Subscribers Only. What is temporal arteritis? Untreated, it can lead to blindness.Prompt treatment with corticosteroid medications usually relieves symptoms … In GCA, involvement of the aorta and its proximal branches is often asymptomatic but may cause vascular bruits or reduced blood pressure in one or both arms. Giant cell arteritis is treated with medications, such as prednisone. GCA affects the blood supply to the scalp, jaw muscles or the back of the eye and is treated with high-dose glucocorticoids (steroids). Table 3 shows examples of symptoms that may signify relapse in patients with GCA and how they might be managed. Patients treated for GCA should be evaluated for features of the disease relevant to prognosis, such as clinical and laboratory features of a marked inflammatory response at diagnosis, ischaemic manifestations such as transient visual loss or jaw/tongue claudication and signs or symptoms indicating involvement of the aorta and its proximal branches and for comorbidities relevant to treatment, such as diabetes mellitus, hypertension and bone fracture risk. QoE: insufficient evidence. It's serious and needs urgent treatment. Strong recommendation: Tocilizumab can be considered for GCA in combination with a glucocorticoid taper, especially in patients at high risk of glucocorticoid toxicity or who relapse. It involved a rigorous process using a framework for evidence appraisal called GRADE, coupled with BSR's guidelines protocol, which is endorsed by NICE. Should I send my patient with previous giant cell arteritis for imaging of the thoracic aorta? Giant-cell arteritis and polymyalgia rheumatica, Permanent visual loss and cerebrovascular accidents in giant cell arteritis: predictors and response to treatment, Prospective long term follow-up of a cohort of patients with giant cell arteritis screened for aortic structural damage (aneurysm or dilatation), BSR and BHPR guidelines for the management of giant cell arteritis, EULAR recommendations for the management of large vessel vasculitis, 2015 Recommendations for the management of polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative, BSR and BHPR guidelines for the management of polymyalgia rheumatica, 2017 American College of Rheumatology guideline for the prevention and treatment of glucocorticoid-induced osteoporosis, GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. It is a critical ischaemic disease and … Consensus score: 9.81. Patients receiving high-dose glucocorticoids are at an elevated risk of osteoporosis and bone fracture; this risk should be managed appropriately. Consensus score: 9.44. This could be either a temporal artery biopsy at least 1 cm in length or an ultrasound of the temporal and axillary arteries, or both. Bhaskar Dasgupta – Paid consultancies for membership on Clinical Trials Advisory Boards, for developing trial protocols as well as speaker fees from Roche-Chugai, Sanofi, ERT, Bristol-Myers Squibb, GlaxoSmithKline and AbbVie as well as ultrasound workshop/GCA symposium grants to Southend University Hospital. Conditional recommendation: Methotrexate might be considered for GCA, in combination with a glucocorticoid taper, in patients at high risk of glucocorticoid toxicity or who relapse. An example of glucocorticoid tapering for GCA, Examples of symptoms that may signify relapse of GCA during glucocorticoid taper that require further evaluation and, if judged to be due to GCA relapse, escalation of glucocorticoid treatment. For doses, see Treatment of GCA, below. The guideline reviews this latest evidence. Patients presenting with a history of new visual loss (transient or permanent) or double vision should be evaluated as soon as possible on the same calendar day by an ophthalmologist. Blood should be taken for full blood count, CRP and ESR before or immediately after commencing high-dose glucocorticoids. It is best practice for the prescriber of glucocorticoid therapy to ensure that patients are evaluated for hypertension and hyperglycaemia (blood glucose for acute changes and/or haemoglobin A1c to identify patients that might be at greater risk) within the first 2 weeks of commencing high-dose glucocorticoids. Patients in whom GCA is strongly suspected should be immediately treated with high-dose glucocorticoids. Garcia-Martinez A, Arguis P, Prieto-Gonzalez S et al. In contrast, other immunosuppressants (including azathioprine, leflunomide and mycophenolate) have not been adequately tested in clinical trials. Fortunately, a new medication called tocilizumab was approved by the Food and Drug Administration in 2017 to treat temporal arteritis. QoE: +. It primarily affects branches of the external carotid artery, and it is the most common form of systemic vasculitis in adults. There have been major developments in the treatment of giant cell arteritis since the last guideline was produced in 2010, particularly with imaging and biologic therapy. GCA is therefore a medical emergency requiring immediate treatment. 4. New visual loss or diplopia should be urgently evaluated by an ophthalmologist. Dr Mackie continues: “This guideline provides a coherent statement of what is the latest best practice. Does this patient have temporal arteritis? If the blood tests are normal, you probably do not have GCA. A positive temporal artery biopsy showing features of inflammation characteristic of GCA, such as giant cells or panarteritis , confirms the diagnosis of GCA. More information on accreditation can be viewed at www.nice.org.uk/accreditation. Your comment will be reviewed and published at the journal's discretion. Justin C. Mason – Speaker fees and consultancy fees from Roche/Chugai. Symptoms may include headache, pain over the temples, flu-like symptoms, double vision, and difficulty opening the mouth. Biologic therapy: Since the previous guidelines, the drug tocilizumab has been licensed for GCA, prescribed alongside steroids for patients who have relapsed, as well as for the small minority who do not respond to initial steroid treatment. 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