BSR and BHPR guidelines for the management of giant cell arteritis

Abstract

GCA is the commonest of all the vasculitides. Visual loss occurs in up to one-fifth of patients, which may be preventable by prompt recognition and treatment [1,2]. The aim of these guidelines is to encourage the prompt diagnosis and management of GCA, with emphasis on the prevention of visual loss. Their scope is to provide evidence-based advice for the assessment and diagnosis of GCA, for initial and further management and for monitoring of disease activity, complications and relapse. This is a summary of the guidelines and the full guideline is available at Rheumatology online. The recommendations for the guidelines are set out in points 1 to 9. (1) Early recognition and diagnosis of GCA is paramount [2]. Particular attention should be paid to the predictive features of ischaemic neuro-ophthalmic complications (C). A patient >50 years of age presenting with the following features should raise suspicion of GCA: Abrupt-onset headache (usually unilateral in the temporal area). Scalp tenderness. Jaw and tongue claudication. Visual symptoms (including diplopia). Constitutional symptoms. Polymyalgic symptoms. Limb claudication. Examination may show: Abnormal superficial temporal artery: tender, thickened with reduced or absent pulsation. Scalp tenderness. Transient or permanent visual loss. Visual field defect. Relative afferent papillary defect. Anterior ischaemic optic neuritis. Central retinal artery occlusion. Upper cranial nerve palsies. Features of large-vessel GCA: vascular bruits and asymmetry of pulses or blood pressure. Laboratory investigations include: Full blood count, urea and electrolytes, liver function tests, CRP, ESR. An acute-phase response is the characteristic of GCA (raised ESR, CRP, anaemia, thrombocytosis, abnormal liver function tests, particularly raised alkaline phosphatase, raised α1 and α2 globulins on serum electrophoresis). However, GCA can occur in the face of lower levels of inflammatory markers, if the clinical picture is typical. Chest radiograph. Urinalysis. Other relevant investigations to exclude mimicking conditions. Features predictive of ischaemic neuro-ophthalmic complications [3, 4]: Jaw claudication. Diplopia. Temporal artery abnormalities. (2) Urgent referral for specialist evaluation is suggested for all patients with GCA. Temporal artery biopsy (TAB) should be considered whenever a diagnosis of GCA is suspected. This should not delay the prompt institution of high-dose glucocorticosteroid therapy (C). TAB can remain positive for 2–6 weeks after the commencement of treatment. It should be performed by a surgical unit experienced in regular TAB, and samples should be at least 1 cm in length. Contralateral biopsy is usually unnecessary. TAB may be negative in some patients. They should be regarded as having GCA if there is a typical clinical picture and response to glucocorticosteroids. (3) Imaging techniques show promise for the diagnosis and monitoring of GCA. However, these do not replace TAB for cranial GCA. Their role in early diagnosis of cranial GCA is an important area of future research (B). The use of duplex ultrasound is currently limited as it requires a high level of experience and training. Other imaging modalities (PET and MRI) should be currently reserved for investigation of suspected large-vessel GCA. (4a) High-dose glucocorticosteroid therapy should be initiated immediately when clinical suspicion of GCA is raised (C). Recommended starting dosages of glucocorticosteroids are: Uncomplicated GCA (no jaw claudication or visual disturbance): 40–60 mg prednisolone daily. Evolving visual loss or amaurosis fugax (complicated GCA): 500 mg to 1 g of i.v. methylprednisolone for 3 days before oral glucocorticosteroids. Established visual loss: 60 mg prednisolone daily to protect the contralateral eye. Patients should also receive bone protection. Proton pump inhibitors for gastrointestinal protection should be considered. The symptoms of GCA should respond rapidly to high-dose glucocorticosteroid treatment, followed by resolution of the inflammatory response. Failure to do so should raise the question of an alternative diagnosis. (4b) Glucocorticosteroid reduction should be considered only in the absence of clinical symptoms, signs and laboratory abnormalities suggestive of active disease (C). This should be balanced against the need to use the lowest effective dose, patient wishes and glucocorticosteroid side effects. Steroid reduction may also be appropriate if the acute-phase response is deemed to be due to another cause. Suggested tapering regimen: 40–60 mg prednisolone continued until symptoms and laboratory abnormalities resolve (at least 3–4 weeks); then dose is reduced by 10 mg every 2 weeks to 20 mg; then by 2.5 mg every 2–4 weeks to 10 mg; and then by 1 mg every 1–2 months provided there is no relapse. The dose may need adjustment for disease severity, comorbid factors, fracture risk, patient wishes and adverse events. There are also some patients w