26th February 2025

Large-vessel vasculitis

There are two main types of primary large-vessel vasculitis: giant cell arteritis (GCA) and Takayasu’s arteritis. Large vessel generally refers to the aorta or its main branches. We will discuss the two main primary causes here; however, there are other secondary causes, such as infection. Clinical features depend on which vessel is involved.

Giant cell arteritis

GCA is the most common form of vasculitis. It generally occurs in middle-aged (>50) Caucasians and has a 2:1 female-to-male ratio. There is an association with polymyalgia rheumatica. This is one of the few rheumatological emergencies, particularly when associated with eye involvement, as there is a very high risk of blindness.

Classical presentation is with a new headache, scalp or temporal artery tenderness, and jaw claudication. Headache can occur anywhere but is most commonly temporal. Visual loss may be present, and this is irreversible. Patients may describe amaurosis fugax as a ‘curtain coming down’ on their vision. The best predictor for future visual loss is previous episodes of visual loss.

Blood tests generally show elevated CRP and ESR. US of the temporal artery is often performed, which may show a ‘halo’ around the artery. Gold standard diagnosis is with temporal artery biopsy; however, a negative biopsy does not rule out the disease, as there is a ‘skip lesion’ pattern, and also, often, patients will have had some treatment by the time they have the biopsy. PET-CT may be performed to look for other areas of large vessel involvement. If there is any suggestion of visual involvement, urgent ophthalmological assessment should be arranged.

Anterior ischaemic optic neuropathy is the most common ocular manifestation of GCA. Posterior ischaemic optic neuropathy can occur but is less common. Central retinal vein occlusion can be seen, but this is much rarer.

Treatment is with high-dose glucocorticoids such as 60 mg oral prednisolone or, if there is visual involvement, 1000 mg IV methylprednisolone for 3 days before stepping down to oral. Steroids can then slowly be weaned over around 12 months, depending on response. Given these patients will be on high-dose steroids for a long period of time, bone protection and gastroprotection should be prescribed. In patients who have steroid-resistant or dependent disease, DMARDs such as methotrexate or biologic agents such as tocilizumab can be used.

Polymyalgia rheumatica (PMR)

Polymyalgia rheumatica as an autoinflammatory condition with an unknown pathophysiology which affects the shoulder and hip girdle. This is not a form of vasculitis; however, it shares some common features with GCA, and 10-20% of patients with PMR will go on to develop GCA. PMR occurs in a similar patient group to GCA, as described above. 

Patients typically present with symmetrical muscle pain and stiffness in the hip and shoulder girdle, which is worst in the morning. This can be so severe it can limit activities of daily living, for example, with tasks that require patients to lift their arms above their head. Systemic symptoms such as malaise, anorexia, and fatigue are common. Low-grade fever can be seen; however, if present, this should prompt thorough investigation to rule out infection. It is important to rule out concomitant GCA when assessing someone presenting with PMR symptoms.

CRP and ESR are almost always raised, and normal values should prompt investigation for an alternative diagnosis. There are no associated autoantibodies.

Treatment is with low-dose prednisolone (15-20 mg), and patients often feel significant symptomatic relief even after one dose. If there is no response to steroids, another diagnosis should be considered. Prednisolone is subsequently weaned slowly.

Takayasu’s arteritis

Takayasu’s is a granulomatous arteritis which affects the aorta and its main branches. It generally affects young women from the ages of 20-40 and is most common in Japan and Southeast Asia. 

Classical findings are manifestations of the consequences of vascular inflammation, such as bruits, claudication and asymmetrical or absent pulses.

Investigation is with cross-sectional imaging and angiography.

Initial treatment is with medium-dose glucocorticoids, such as 40-60 mg prednisolone, and then DMARDs, such as methotrexate, azathioprine, or MMF, if the features are steroid-resistant.