Giant cell arteritis and polymyalgia rheumatica

• Giant cell arteritis (GCA) is a granulomatous large-vessel vasculitis that usually begins after the age of 50 years. It inflames the aorta, its branches and often the cranial arteries, and can destroy sight in one or both eyes within days.
• Its signature clues are a new persistent headache, scalp or temporal artery tenderness, jaw claudication on chewing, and visual symptoms, with a markedly raised erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP).
• Suspect it, start high-dose glucocorticoids the same day, and arrange confirmatory tests afterwards. Vision already lost may not return, but treatment protects the other eye.
• Temporal artery biopsy, ultrasound and vascular imaging confirm the diagnosis; tocilizumab is the main glucocorticoid-sparing biologic in relapsing or high-risk disease.
• Polymyalgia rheumatica (PMR) is the closely related inflammatory syndrome of the shoulder and hip girdles in older adults. It often starts before or with GCA and is treated with lower-dose glucocorticoids.
• Takayasu arteritis is the sister disease of the aorta and its branches in younger patients, managed with glucocorticoids plus a non-glucocorticoid immunosuppressant from the start.

By the end of this page you should be able to:

1. Define giant cell arteritis and polymyalgia rheumatica, place them among the large-vessel vasculitides, and distinguish them from Takayasu arteritis.
2. Explain GCA from its immunology to the bedside: why the artery hurts, why the jaw stops, why vision fails.
3. Recognise the cranial, large-vessel and PMR-overlap phenotypes of GCA and pick the urgent red flags.
4. Choose and interpret ESR and CRP, temporal artery biopsy, ultrasound, CT or MR angiography and PET in suspected GCA.
5. Start treatment with the right glucocorticoid dose and route, add tocilizumab or methotrexate appropriately, and taper without precipitating relapse.
6. Manage polymyalgia rheumatica with its own dose, adjust for older adults, and outline surveillance and prognosis.

GCA, PMR and Takayasu arteritis sit under the heading of large-vessel vasculitis (LVV): inflammation of the aorta and its large branches, with or without cranial involvement. In GCA the inflamed artery is typically the aorta, subclavian, axillary, carotid and vertebral arteries and the superficial temporal artery; in PMR the problem is the peri-articular and bursal inflammation around the shoulder and hip girdles; in Takayasu the disease centres on the aorta and the branch vessels.

Two phenotypes of GCA matter in practice. Cranial GCA presents with headache, scalp tenderness and visual symptoms. Large-vessel GCA affects the aorta and its branches, with less headache and visual loss but more limb claudication and PMR symptoms. The two overlap; one patient can have both, and large-vessel involvement is found in many patients once vascular imaging is added.

GCA is the commoner of the two diseases that define this topic. The pooled incidence among adults aged 50 years or older is 10.0 to 15.5 per 100,000, highest in Scandinavia and lowest in Asia, where the same figure is about 0.34 per 100,000.^[3] Incidence peaks at 70 to 79 years of age, and the disease is almost absent before 50.^[3] Women are affected about two to four times as often as men.^[3] The prevalence is rising with population ageing, which makes GCA an increasingly important cause of headache and visual loss in older adults.

Takayasu arteritis is rare worldwide: its overall incidence is about 1.29 per million person-years, higher in Asia than in Europe.^[3] Prevalence peaks above 40 per million in Japan and reaches about 33 per million in Türkiye, while most European regions report 4 to 25 per million.^[3] It usually begins before the age of 40 years.^[3]

PMR is commoner than GCA in older adults. In one Asian claims-based analysis, annual incidence was 2.06 per 100,000 in people aged 50 years or older and prevalence 8.21 per 100,000, with an age and sex distribution resembling Western cohorts.^[3] PMR occurs in roughly 40 to 60% of patients with GCA at disease onset or during relapse, so the two are not separate stories.^[3]

Risk factors include age, female sex and ethnicity. Genetic associations differ: GCA carries an association with HLA-DRB104, whereas Takayasu arteritis is linked to HLA-B52:01.^[3] Both share variation at IL12B, which encodes the p40 subunit shared by IL-12 and IL-23 and therefore influences Th1 and Th17 differentiation.^[3] Modifiable risk factors are not established; GCA is not explained by smoking, infection or lifestyle in the available evidence.

Several features at presentation predict permanent visual loss or relapse, so they are collected early. In a systematic review informing the EULAR 2018 recommendations, jaw claudication, headache, temporal artery tenderness, transient visual ischaemic symptoms, cerebrovascular symptoms and an increased platelet count were associated with visual complications.^[5] By contrast, coexistent PMR, upper-limb artery involvement, constitutional symptoms, an elevated CRP and low haemoglobin conferred a lower risk.^[5] Relapses are more common in the first year after diagnosis, especially in patients with scalp tenderness, PMR, or a strong inflammatory response defined as three or more of the following: fever above 38 °C, weight loss of 4 kg or more, haemoglobin under 11 g/L, and an ESR of 85 mm/h or more.^[5]

GCA begins in the wall of the large artery itself. The arterial wall is normally immune-privileged, with only sparse vascular dendritic cells at the junction of adventitia and media. In GCA those cells are activated by danger signals, present antigen and engage CD4+ T cells, which differentiate into Th1 and Th17 cells. Th1 cells release interferon-gamma and tumour necrosis factor-alpha; Th17 cells release IL-17 and promote giant-cell formation. Activated macrophages over-express MMP-9, breaking down elastic laminae, and release IL-1, IL-6, PDGF, GM-CSF and VEGF. The result is granulomatous inflammation, intimal hyperplasia, luminal narrowing and, in some, aneurysmal dilatation.^[3]

Visual symptoms follow occlusion or severe stenosis of the arteries supplying the optic nerve or retina, which is why they can appear suddenly and progress rapidly. Jaw claudication reflects ischaemia of the masseter muscles, tongue claudication reflects ischaemia of the tongue, and scalp tenderness reflects inflammation and ischaemia of the superficial temporal artery and overlying skin. Constitutional symptoms and a raised ESR or CRP arise from the systemic release of IL-6.

Several drugs now target these pathways. Tocilizumab blocks IL-6 signalling. TNF-alpha inhibitors, IL-12/23 and IL-17 blockers and small molecules are under study, particularly where the Th1 axis proves steroid-resistant.

The distinction between GCA and Takayasu arteritis is not only age and geography. GCA lesions are dominated by CD4+ T cells and occupy the intima and inner media. Takayasu lesions show more prominent CD8+ T cells and natural killer cells, with marked adventitial expansion, collagen-rich remodelling and late fibrosis.^[3] The IL-6 and Th17 axis in GCA is usually glucocorticoid-responsive, which is why steroids work quickly, whereas Th1-driven disease can be chronic.^[3]

PMR's mechanism is less clear from the current evidence. It is an inflammatory syndrome of the shoulder and hip girdles that shares an age band and an IL-6-driven inflammatory profile with GCA; it precedes, accompanies or follows GCA. Histology of the girdle tissues shows synovitis and bursitis rather than arterial vasculitis.

The classic cranial presentation is a new headache that is persistent, often temporal or frontal, and different from any previous headache. Scalp tenderness can make a comb or pillow painful, and jaw claudication, pain or fatigue of the masseter on chewing, is a specific but not universal clue. Tongue claudication and throat pain on swallowing are less common.

Visual symptoms define the emergency: transient monocular visual loss (amaurosis fugax), diplopia, and sudden permanent visual loss, which is most often anterior ischaemic optic neuropathy or central retinal artery occlusion. Constitutional features are fatigue, malaise, low-grade fever, night sweats and weight loss. Polymyalgia symptoms are present in 40 to 60% of patients at onset or at relapse.^[3]

Large-vessel GCA has a different emphasis: constitutional symptoms and arm or leg claudication, with less headache and less visual loss.^[5]

The patient, usually aged 50 years or older, reports bilateral aching and morning stiffness of the shoulders, neck and hips, worse on rising and improving during the day. There is no weakness on testing. Symptoms often appear abruptly, sleep is disturbed, and the ESR or CRP is raised. GCA symptoms must be sought explicitly, because PMR and GCA sit on the same spectrum and one can become the other.

Younger patients present with limb claudication, fatigue, absent or unequal pulses, blood-pressure differences between arms, vascular bruits, hypertension when the renal arteries are involved, dizziness or syncope, and visual symptoms from ischaemia. Constitutional symptoms are common during active disease.

Look at the temporal artery for tenderness, thickening or loss of pulsation, and listen for bruits over the carotids, subclavians and abdomen. Check all peripheral pulses and record blood pressure in both arms. Examine the eyes and fundi and the visual fields when vision is affected, and use slit-lamp or formal visual fields when possible. In PMR test shoulder and hip movement and look for a normal muscle strength, which distinguishes inflammatory pain from true myopathy.

ESR and CRP are the workhorse markers. Both are raised in most patients with active GCA, sometimes dramatically, and they are used to follow response. A complete blood count commonly shows anaemia and thrombocytosis; platelets may be high. Liver enzymes are often mildly raised.

Biopsy of the superficial temporal artery remains the reference test in many settings and provides a histological diagnosis. ACR 2021 recommends a unilateral, long-segment biopsy of more than 1 cm rather than a short segment, because longer samples improve yield.^[2] Biopsy should be performed within two weeks of starting oral glucocorticoids, since histological changes persist but become patchier after that.^[2] Studies that inform EULAR 2018 reported sensitivity of about 39% against clinical diagnosis with 100% specificity for temporal artery biopsy, which means a negative biopsy does not exclude disease and imaging and clinical judgement still matter.^[5]

Histology shows granulomatous inflammation, destruction of the internal elastic lamina and, in many cases, multinucleated giant cells. Their absence does not exclude the diagnosis.^[3]

Temporal and axillary artery ultrasound can show a non-compressible hypoechoic halo around the vessel wall, stenoses and occlusions. It is operator dependent and results are quickly altered by glucocorticoids. In centres with trained operators it complements biopsy; ACR 2021 still conditionally prefers biopsy in the United States because of variable ultrasound expertise.^[2]

Non-invasive vascular imaging is recommended in newly diagnosed GCA to look for large-vessel involvement, and regularly scheduled imaging is recommended in Takayasu arteritis.^[2] CT angiography, MR angiography, duplex ultrasound and PET-CT each have roles: CT and MRA define stenoses, dilatations and dissections; PET-CT shows metabolically active large-vessel inflammation, particularly in the aorta and proximal branches.

ECG and chest imaging are used to seek large-vessel or cardiac complications. In Takayasu arteritis, measures of renal function and blood pressure in all limbs, echocardiography of the aortic root and valve, and carotid and subclavian imaging are routine.

Formal classification criteria exist for both diseases and are revised from time to time. In practice, the diagnosis is clinical and supported by raised inflammatory markers and imaging or biopsy. The EULAR consensus definitions used for trials and registries are useful when classifying disease activity. Active disease requires typical signs or symptoms of large-vessel vasculitis plus at least one of: current activity on imaging or biopsy, ischaemic complications attributed to the vasculitis, or persistently raised inflammatory markers after other causes have been excluded. Major relapse is recurrence with ischaemic features such as jaw claudication, visual symptoms, visual loss, stroke or limb claudication, or progressive aortic dilatation, stenosis or dissection. Minor relapse is recurrence without those features. Remission is the absence of clinical signs or symptoms of active disease with normal ESR and CRP and, for extracranial disease, no progressive vessel narrowing or dilatation. Sustained remission is remission lasting at least six months with achievement of the individual target glucocorticoid dose, and glucocorticoid-free remission is sustained remission with glucocorticoids stopped while other immunosuppressive treatment may continue.^[1]

The overriding rule is to start high-dose glucocorticoids on clinical suspicion, especially if there are visual symptoms, and not to wait for the biopsy, imaging or laboratory results.^[4] Fast-track pathways that bring the patient to a specialist within 24 hours and integrate ultrasound reduce permanent visual loss compared with usual care.^[1][5]

For GCA, EULAR 2018 and the BSR 2020 guideline recommend starting treatment with 40 to 60 mg of prednisolone-equivalent per day.^[1][4] The 2025 EULAR revision keeps the same 40 to 60 mg range for GCA or Takayasu arteritis.^[4] ACR 2021 recommends high-dose oral glucocorticoids over moderate dose for newly diagnosed GCA.^[2]

In a patient without cranial ischaemia, high-dose oral glucocorticoids are preferred over intravenous pulses.^[2] In threatened vision loss, intravenous pulse glucocorticoids are preferred over high-dose oral therapy.^[2] EULAR recommends 0.25 to 1 g of intravenous methylprednisolone daily for up to three consecutive days when there is acute visual loss or amaurosis fugax, although the evidence is limited.^[1] Routine intravenous pulses are not recommended for patients without visual impairment.^[1]

Glucocorticoids should be given once daily, not on alternate days and not split into multiple daily doses.^[1][2]

A workable taper, supported by EULAR and the trial data behind tocilizumab, is to reach 15 to 20 mg per day within 2 to 3 months and 5 mg per day or less by 1 year.^[1] Relapse is rare at a dose above 20 mg per day.

Most patients eventually come off glucocorticoids; indeed, about 30 to 60% of patients do not relapse and reach 5 mg per day or less at 1 year.^[1] EULAR and BSR advise that patients without risk factors for glucocorticoid adverse effects or a prolonged course may be treated with glucocorticoids alone.^[1]

Glucocorticoids are both the treatment and the main long-term hazard. Bone protection, blood-pressure control, glucose monitoring, infection avoidance and (where available) vaccination are part of the prescription, not optional extras. ESR and CRP are used to follow disease activity, but structural vascular disease and PMR symptoms can also drive management, and glucocorticoid-sparing agents allow lower cumulative steroid exposure.

Tocilizumab, an IL-6 receptor blocker, is the best established steroid-sparing agent. ACR 2021 recommends oral glucocorticoids with tocilizumab over glucocorticoids alone in newly diagnosed GCA, and the 2025 EULAR recommendation offers IL-6 receptor inhibitors as adjunctive therapy for selected patients with PMR or GCA.^[2][4] In the pivotal trial 162 mg of tocilizumab weekly or every other week subcutaneously, combined with a 26-week glucocorticoid taper, achieved sustained glucocorticoid-free remission at 52 weeks in 56% and 53% of patients respectively, versus 14% and 18% with glucocorticoids alone.^[1]

Methotrexate is an alternative adjunct. Evidence from a meta-analysis of three placebo-controlled studies shows that it reduces first and second relapses and increases the probability of glucocorticoid-free remission for at least 24 weeks, although one of the trials used only 7.5 mg weekly and was small.^[1] ACR 2021 recommends adding a non-glucocorticoid immunosuppressive drug in GCA that relapses on moderate or high-dose glucocorticoids, and adding tocilizumab rather than methotrexate when cranial ischaemia recurs.^[2]

Aspirin should be added for patients with critical or flow-limiting involvement of the vertebral or carotid arteries.^[2] Statins are not recommended specifically for GCA.^[2]

For patients with active extracranial large-vessel involvement, oral glucocorticoids combined with a non-glucocorticoid immunosuppressive agent are preferred to glucocorticoids alone.^[2]

ACR 2021 recommends high-dose glucocorticoids rather than low-dose for newly active severe disease, and starting with high-dose oral rather than pulse intravenous therapy in severe active disease not already on immunosuppression.^[2] A non-glucocorticoid immunosuppressive agent is recommended with glucocorticoids from the outset, rather than glucocorticoids alone, using methotrexate, azathioprine or a TNF-alpha inhibitor as initial therapy.^[2] Tocilizumab is not recommended as initial therapy because it failed to meet its primary endpoint in the only randomised trial in Takayasu arteritis; if disease is refractory to glucocorticoids alone, a TNF-alpha inhibitor is preferred over tocilizumab.^[2]

Glucocorticoids can be tapered off once remission has lasted 6 to 12 months rather than continuing low-dose glucocorticoids indefinitely.^[2] Aspirin or another anti-platelet agent is added for active disease with critical cranial or vertebrobasilar involvement.^[2] Surgery is delayed until the disease is quiescent if there are worsening signs of limb or organ ischaemia.^[2] High-dose glucocorticoids are given in the peri-procedural period if the disease is active.^[2]

PMR is treated with lower induction doses than GCA: 15 to 25 mg of prednisone-equivalent per day, as stated in the 2025 EULAR recommendation.^[4] Taper is slow, often over many months, with relapse on rapid taper being common. Adjunctive treatment for selected patients includes an IL-6 receptor inhibitor for PMR or GCA, or upadacitinib for GCA, with methotrexate as an alternative.^[4] The clinician should monitor for the emergence of GCA throughout. PMR also responds promptly to glucocorticoids; a response within days is expected and can support the diagnosis. Long-term, the main challenge is distinguishing relapse from other causes of pain, and avoiding prolonged high-dose exposure.

In settings where vascular imaging, PET-CT or a specialist is hard to reach, the diagnosis must still be clinical: age over 50, new headache, jaw claudication, scalp tenderness and raised ESR or CRP, with ESR and CRP available almost everywhere. Start prednisolone and refer. Temporal artery biopsy can be delayed up to two weeks. A simple temporal artery ultrasound by a trained operator is cheaper than biopsy; if neither is available, treat and refer. The slow glucocorticoid taper demands reliable follow-up, so prescribe bone protection and review regularly.

• Permanent visual loss, the most feared complication of GCA, most often from anterior ischaemic optic neuropathy or central retinal artery occlusion.
• Stroke, especially posterior circulation, and other cerebrovascular events.
• Large-vessel disease: stenosis, occlusion, aneurysm and dissection of the aorta and its branches, which is commoner in LV-GCA and in Takayasu arteritis.
• Relapse, most frequent in the first year after diagnosis.
• Glucocorticoid harm: osteoporosis and fractures, diabetes, hypertension, infection, weight gain and adrenal suppression.
• In Takayasu arteritis: resistant hypertension from renal artery stenosis, limb or organ ischaemia, aortic regurgitation and pregnancy complications.

Glucocorticoids control GCA quickly and the short-term outlook is excellent once treatment starts, but relapse requires prolonged therapy. Fast-track diagnosis reduces permanent visual loss.^[1][5] Large-vessel involvement carries more long-term risk: in one cohort of GCA patients followed for a median of 10.3 years, aortic structural damage developed in about one-third, defined as focal dilatation or a thoracic aortic diameter of at least 4 cm or an abdominal aortic diameter of at least 3 cm.^[5] Such damage is associated with increased mortality in GCA.^[5] Surgical repair is only feasible in about one-third of those affected because of age and comorbidity.^[5]

Takayasu arteritis tends to run a relapsing course, with vascular restenosis despite therapy. Outcomes improve with early immunosuppression and regular non-invasive imaging.

There is no primary prevention. Once GCA or Takayasu arteritis is diagnosed, screening for large-vessel complications with vascular imaging at baseline and follow-up is recommended.^[2] In GCA, monitor blood pressure and lipids, protect bone with calcium, vitamin D and antiresorptive therapy as indicated, and review infection prevention in line with standard care. Long-term clinical monitoring is strongly recommended for GCA in apparent remission and for Takayasu arteritis.^[2]

Older adults are the population in whom GCA, PMR and the vascular complications of both are most common. Comorbidity and frailty modify the glucocorticoid dose and the choice of immunosuppression, and bone, glucose and blood-pressure monitoring are essential.

Children and adolescents almost never develop GCA. Takayasu arteritis can begin in childhood and adolescence and shares the same management principles.

Pregnancy is uncommon in GCA because of age, but Takayasu arteritis often affects women of childbearing age. Pre-conception counselling, pregnancy-compatible immunosuppression and avoidance of contraindicated agents such as methotrexate are required, with care shared between rheumatology, obstetrics and vascular medicine.

Plain-text related topics: fever of unknown origin; secondary headache disorders; acute kidney injury; retinal vascular occlusion; rheumatoid arthritis; osteoporosis and glucocorticoid-induced bone disease. Read these for context.

• GCA is a granulomatous large-vessel vasculitis of people aged 50 years or more; cranial and large-vessel phenotypes overlap.
• Suspicious older patients need ESR, CRP, temporal and large-vessel assessment, and high-dose glucocorticoids the same day when suspicion is strong, especially with visual symptoms.
• Classical clues are a new persistent headache, scalp tenderness, temporal artery thickening, jaw claudication and visual symptoms; PMR coexists in 40 to 60%.
• ESR and CRP are usually raised; a normal marker does not replace careful clinical assessment.
• Temporal artery biopsy of a segment longer than 1 cm, ideally within two weeks of starting steroids, supports the diagnosis; sensitivity against clinical diagnosis is about 39% and specificity 100%.
• Ultrasound, CT or MR angiography and PET-CT define cranial and large-vessel involvement; repeat imaging follows aortic complications.
• Induction dose in GCA is 40 to 60 mg prednisolone-equivalent daily; 0.25 to 1 g intravenous methylprednisolone daily for up to three days if there is visual loss.
• Taper to 15 to 20 mg daily by 2 to 3 months and 5 mg or less by 1 year; 30 to 60% of patients never relapse.
• Tocilizumab is the main steroid-sparing biologic in GCA; methotrexate and aspirin have defined roles; statins are not GCA therapy.
• PMR is treated with 15 to 25 mg prednisone-equivalent daily, tapered slowly over months, with IL-6 inhibitors or methotrexate as adjuncts.
• Takayasu arteritis begins before 40 years and is treated with high-dose glucocorticoids plus a non-glucocorticoid immunosuppressant, with TNF-alpha inhibitors preferred over tocilizumab for refractory disease.
• Fast-track referral, bone protection and long-term vascular imaging are part of standard care.