Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with giant cell arteritis
Norwegian Society of Rheumatology · 2023 · Europe
Source and licence
- Authors
- Anne Bull Haaversen, Lene Kristin Brekke, Gunnstein Bakland et al.
- Title
- Norwegian society of rheumatology recommendations on diagnosis and treatment of patients with giant cell arteritis
- Journal
- Frontiers in Medicine, 2023
- Licence
- CC BY 4.0
- Changes
- Verbatim excerpts; formatting adapted
Full text retrieved 2026-10-27 from Europe PMC · PMC9853546
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Recommendations
Verbatim, with grade of recommendation or strength of consensus where given.
Diagnosis
- Recommendation 1In patients with suspected GCA, a thorough history should be obtained, including inquiry about polymyalgia symptoms, new-onset headache, tongue- or jaw-claudication, vision disturbances, arm or leg claudication, constitutional symptoms (fatigue, fever, and weight loss). Additionally, a thorough clinical examination and laboratory workup should be performed including heart and lung auscultation, blood pressure in both arms, temperature, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and full blood count (Table 1).
- Recommendation 2Patients suspected of having GCA, should be directly referred to a Fast-Track Clinic (FTC) or a rheumatologist for further evaluation, treatment, and follow-up within 24 h. In cases where FTC- or rheumatology consult is unavailable within 24 h the patient may be referred to other relevant specialists (e.g., neurologist, ophthalmologist, and internist). FTC consists of an evaluation by ultrasound or other imaging modality which can confirm the diagnosis immediately (19). Diagnostic work-up should not delay the initiation of treatment.
- Recommendation 3In patients with suspected GCA, ultrasound of at least temporal and axillary arteries (20) should be performed by an ultrasonographer experienced in vascular ultrasound using high-end ultrasound equipment (21) (Tables 2, 3). Ultrasound of the facial artery further increases the sensitivity to diagnose GCA (20). If ultrasound is not available or inconclusive, a biopsy of the temporal artery should be considered. The length of the biopsy should be >1 cm after fixation. Giant cells in the biopsy are not obligate for the diagnosis of GCA, but appropriate pathological findings should be present (Table 4). Alternatively, Magnetic Resonance Angiography (MRA) of temporal arteries or Positron Emission Tomography (PET-CT) may be performed. In addition, ultrasound of large vessels (carotid, vertebral, and subclavian) (22) or CT of large vessels or MRA or PET-CT depending on the availability is recommended. It is essential to recognize the disease extent as early as possible as large vessel involvement may indicate difficult-to-treat disease, while temporal artery involvement has been associated with a higher risk for visual loss (23, 24). The choice of imaging modality depends on the local availability.
- Recommendation 4In a patient with high clinical suspicion of GCA and a positive diagnostic test (biopsy or imaging modality), no further test is required to confirm the diagnosis. In patients with low clinical suspicion of GCA and a negative diagnostic test (biopsy or imaging modality), the probability for GCA is low. In other cases, an individual assessment will be necessary for further diagnostics.
- Recommendation 5In patients with GCA and visual symptoms, referral to an ophthalmologist is highly recommended. Initiation of treatment should not be delayed while waiting for this evaluation (Figure 1 and Table 5).
Treatment
- Recommendation 6In patients with suspected GCA, glucocorticoids should be immediately initiated. The diagnostic work-up should not delay the initiation of treatment. For patients without visual manifestations we recommend a starting dose of 40 mg Prednisolone/day. In patients with visual involvement (visual loss, diplopia, amaurosis fugax, and blurred vision), we recommend starting with 60 mg Prednisolone/day. A single dose of 500 mg × 1 methylprednisolone iv followed by Prednisolone 60 mg/day may be individually considered, but the evidence is sparse (25–27).
- Recommendation 7In patients with GCA, Prednisolone should be tapered by 5 mg every 2nd week till 20 mg/day, after that with 2.5 mg every 3rd week till 10 mg/day, and after that with 1.25 mg every 3rd week to 5 mg/day. We recommend continuing treatment with 5 mg/day at least for 1 year after initiation of Prednisolone. Further tapering can be considered on an individual basis if the patient has been in remission for at least 1 year. When starting with dose 60 mg/day, Prednisolone should be tapered by 10 mg every week till 40 mg/day, thereafter tapering as described above (Table 6).
- Recommendation 8If the patients suffers a relapse, we recommend an increase in Prednisolone dose to the last effective dose, or a higher dose based on the severity of the relapse and an individual assessment. In patients with refractory disease or a major relapse (Table 7), initiation of Methotrexate (MTX), preferably subcutaneously, 20 mg/week, should be considered. The dose should be adjusted according to the patient’s age and kidney function. Tocilizumab (TCZ) 162 mg/week sc should be considered if the patient is not tolerating MTX or suffer a relapse while on MTX (according to the Norwegian Tender System). Leflunomide or Azathioprine may be considered, but evidence supporting their use is scarce. There is currently no robust evidence supporting the use of TNF-α inhibitors or other biologics than TCZ in patients with GCA.
- Recommendation 9Giant cell arteritis patients with new-onset disease should be referred to a measurement of bone mass density. In patients with GCA and high risk for osteoporosis, treatment according to the Norwegian guidelines for osteoporosis should be initiated (28).
- Recommendation 10In patients with GCA, we do not routinely recommend using Acetylsalicylic acid unless cardiovascular reasons support its use (Figure 2).
Follow up
- Recommendation 11In GCA patients, follow-up should be continued every month until remission is achieved. Thereafter follow-up at 3 months, 6 months, and thereafter yearly.
- Recommendation 12In GCA patients who suffer a relapse, the relapse should, if possible, be confirmed by an imaging modality, preferably ultrasound, and/or laboratory tests (29, 30). Modified Kerr’s (NIH) criteria (originally developed for Takayasu arteritis) may be used to monitor disease activity (31) (Tables 7, 8).
- Recommendation 13In GCA patients with relapsing or refractory disease, alternative diagnoses should be considered (e.g., malignancy, autoinflammatory syndromes).
Abstract
Objective: To provide clinical guidance to Norwegian Rheumatologists and other clinicians involved in diagnosing and treating patients with giant cell arteritis (GCA).
Methods: The available evidence in the field was reviewed, and the GCA working group wrote draft guidelines. These guidelines were discussed and revised according to standard procedures within the Norwegian Society of Rheumatology. The European Alliance of Associations for Rheumatology (EULAR) recommendations for imaging and treatment in large vessel vasculitis and the British Society for Rheumatology (BSR) guidelines for diagnostics and treatment in GCA informed the development of the current guidelines.
Results: A total of 13 recommendations were developed. Ultrasound is recommended as the primary diagnostic test. In patients with suspected GCA, treatment with high doses of Prednisolone (40–60 mg) should be initiated immediately. For patients with refractory disease or relapse, Methotrexate (MTX) should be used as the first-line adjunctive therapy, followed by tocilizumab (TCZ).
Conclusion: Norwegian recommendations for diagnostics and treatment to improve management and outcome in patients with GCA were developed.
Contents of the original publication
The section headings of the original.
- Introduction
- Background
- Objectives of the guidelines
- Methods
- Results
- Diagnosis
- Treatment
- Follow up
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