Introduction
Who are affected?
Making a diagnosis
Treatment
Prognosis
Complications
Conclusion
References

 

 

Introduction

Giant cell arteritis is a disease characterised by inflammation of large and medium-sized blood vessels. An alternative name for this condition is temporal arteritis as the blood vessels in the temple area of the head (sides of the forehead) are commonly affected. The giant cells referred to are specific collections of immune system cells seen in the areas of inflammation if a biopsy is performed.2

It involves the major branches of the aorta with predilection for the extracranial branches of the carotid artery. It occurs in individuals older than 50 years and the incidence increases with age. The signs and symptoms of giant cell arteritis can be classified into four subsets: cranial arteritis, extracranial arteritis, systemic symptoms and polymyalgia rheumatica.3

 

Who are affected?

This condition is one of the commonest forms of vasculitis, affecting one or two persons per 10,000 people in the UK. It is more commonly seen in older patients and is seldom diagnosed below the age of 50 years.

GCA is quite often associated with a condition called polymyalgia rheumatica (PMR), which causes severe stiffness of the muscles, particularly in the shoulders and hips. About 15% of PMR patients also develop GCA, while 40–50% of people with GCA also have symptoms of PMR.4

 

Making a diagnosis

As with most types of vasculitis there is no one test that will give the diagnosis. The only test that confirms GCA diagnosis is a temporal artery biopsy, showing vasculitis with mononuclear cell inflammatory infiltrates, often with giant cells. Due to the focal and segmental nature of the infiltrates, areas of inflammation may be missed by the biopsy and the histological examination is normal in about 15% of the cases.

Some imaging modalities may aid in the diagnosis of GCA. Among those, color duplex ultrasonography of the temporal arteries is more commonly used.3

A diagnosis often only occurs if the physician recognises the typical collection of symptoms and clinical signs.

The clinical features of GCA can be classified into: (1) the systemic manifestations of malaise, weight loss, fever, night sweats and depression; (2) the proximal muscle pain and stiffness of polymyalgia rheumatica; (3) arteritic manifestations of pain or tenderness due to local inflammation; and (4) arteritic manifestations of ischaemia due to narrowing or occlusion of vessels. These may occur singly or in any combination and may come and go with the passage of time. Thus GCA can result in many different clinical signs and symptoms.5

 

Treatment

The two aims of treatment are to relieve patients’ symptoms and prevent damage to organs (especially the eyes) due to reduced blood supply. High dose oral steroids (prednisolone) are usually given and the dose is reduced slowly over months, using the erythrocyte sedimentation rate (ESR) marker as a guide to response. Treatment with steroids is usually given for at least one or two years.2

Almost all patients experience side effects from prednisone. After the patient improves, the doctor gradually reduces the prednisone dose. The rate of tapering prednisone depends on how the patient feels, what the doctor finds on exam, and the results of blood tests, including the sedimentation rate. Although virtually all patients are able to reduce their prednisone dose, most require some amount of prednisone for 1–2 years. Longer treatment periods are not uncommon.1

In some patients with resistant or problematic disease other drugs are sometimes used such as methotrexate, azathioprine or antibody therapy such as infliximab. While some patients may respond well to these drugs, there is no current evidence that they are effective in all patients.2

 

Prognosis

There is a relapse rate of about 50%. Withdrawal of the steroids can lead to a relapse of the symptoms. In such cases the steroid is re-started and it is often sufficient to induce remission. The steroid dose can again be reduced. In most relapsed cases the patient will be maintained on a small maintenance dose of steroids indefinitely. The prognosis is good with correct and early diagnosis and treatment.

 

Complications

Damage to the eyes is usually the most serious complication. Involvement of the ophthalmic artery and its branches results in visual loss, which is often complete but is usually painless. Visual loss may be monocular or binocular developing simultaneously or sequentially. Rarely, it stems from occipital lobe infarct that result in homonymous hemianopia, a visual field defect involving the two identical halves (right or left) of the visual fields of both eyes. Visual hallucinations and diplopia are less common. All visual symptoms, including those that are transient, require urgent ophthalmological evaluation and treatment with high-dose glucocorticoids to avoid permanent visual loss.6

Before the advent of glucocorticoid treatment, the prevalence of visual complications was high. Increasing awareness by physicians of the symptoms of GCA and advances in diagnostic techniques over the past 20 years have also contributed to a substantial decline in the frequency of permanent visual loss.7

Ischaemic brain lesions are less common than visual lesions, and mostly result from vasculitis of the extradural vertebral or carotid arteries. In the case of both the eye and the brain, ischaemic damage is thought to result from arterial stenosis or occlusion that occurs secondary to the inflammatory process.7

Another complication highlighted in the case study is scalp ulceration. Scalp necrosis associated with giant cell arteritis was first described in the 1940s. The presence of this dermatological sign within giant cell arteritis represents a severity marker of this disease, with a higher mean age at diagnosis, an elevated risk of vision loss and tongue gangrene, as well as overall higher mortality rates, in comparison to patients not presenting this manifestation. Even though scalp necrosis due to giant cell arteritis is exceptional, a high level of suspicion must be held for this clinical finding, in order to initiate prompt and proper treatment and avoid blindness.8

 

Conclusion

Giant cell arteritis can begin suddenly or gradually with non-specific symptoms. Doctors should have a high degree of suspicion if a patient presents with any combination of the following symptoms: malaise, weight loss, depression, and fatigue or with the classic symptoms of headache, scalp tenderness, jaw claudication, visual changes, or polymyalgia rheumatica.

 

A case of bilateral frontal scalp ulceration

Case study

A 73-year-old man was referred to the dermatology skin cancer clinic with a three-week history of scalp erythema and tenderness followed by bilateral painful frontal scalp ulceration. Jaw claudication was reported on direct enquiry and there were no visual or systemic symptoms.

Physical examination revealed bilateral markedly tender frontal scalp ulceration (Figure 1a) with non-pulsatile temporal arteries. Ophthalmology examination excluded optic neuropathy.

Blood work showed an erythrocyte sedimentation rate (ESR) of 21mm/h.  Histology from temporal artery biopsy was compatible with a diagnosis of giant cell arteritis (GCA) with almost complete occlusion of the vessel lumen with thrombus (Figure 1b).

The patient received oral prednisolone 60mg daily and aspirin 75mg daily. There was a rapid improvement of the scalp tenderness and erythema and gradual healing of the ulceration.

Physicians should be aware that, although uncommon, scalp ulceration can be the main presenting feature of giant cell arteritis. Scalp ulceration is associated with a 32% incidence of visual loss1 and an increased mortality compared to patients without scalp ulceration.1

ESR can be normal in 4% of patients with GCA.2 Early recognition and prompt treatment of the condition is critical in preventing visual loss and other complications.

 

1. Tsianakas A. et al. Scalp necrosis in giant cell arteritis: Case report and review of the relevance of this cutaneous sign of large vessel vasculitis. JAAD 2009; 61(4): 701-706

2. Barraclough K, Mallen C D, Helliwell T, Hider SL, Dasgupta B. Diagnosis and management of giant cell arteritis. Br J Gen Pract 2012; 62(599): 329–30

 

 

Figure 1a. Clinical Photograph

 

 

Figure 1b. Histology shows transmural inflammation of the right temporal artery with marked intimal thickening and oedema. The lumen is almost completely obliterated by thrombus. (H-E stain, amplification x100)

 


Emily Rudd, Department of Dermatology, Chelsea and Westminster Hospital, London

Justin Weir, Department of Histopathology, Imperial College London NHS Healthcare Trust, London

Eirini Merika, Department of Dermatology, Chelsea and Westminster Hospital, London

 

Alison Bloomer, Managing editor, GM

Conflict of interest: none declared

 


References

1. https://www.hopkinsvasculitis.org/types-vasculitis/giant-cell-arteritis/ (accessed 31/07/18)

2. http://www.vasculitis.org.uk/about-vasculitis/giant-cell-arteritis-temporal-arteritis (accessed 31/07/18)

3. Nesher G. The diagnosis and classification of giant cell arteritis. J Autoimmun 2014: 73-5

4. https://www.arthritisresearchuk.org/arthritis-information/conditions/giant-cell-arthritis/who-gets-it.aspx (accessed 31/07/18)

5. Jones JG. Clinical features of giant cell arteritis. Baillieres Clin Rheumatol 1991; 5(3): 413–30

6. Vodopivec I, Rizzo JF.  Ophthalmic manifestations of giant cell arteritis. Rheumatology (Oxford) 2018;57(suppl_2): ii63-ii72

7. Soriano A, Muratore F, Pipitone N, et al. Visual loss and other cranial ischaemic complications in giant cell arteritis. Nat Rev Rheumatol 2017; 13(8): 476–84

8. Maidana DE, Muñoz S, Acebes X, et al. Giant cell arteritis presenting as scalp necrosis.  Scientific World Journal 2011; 11: 1313–15