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  • Review Article
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Visual loss and other cranial ischaemic complications in giant cell arteritis

Nature Reviews Rheumatology volume 13, pages 476–484 (2017)Cite this article

Subjects

Key Points

  • Visual loss is the most feared manifestation of giant cell arteritis (GCA) and occurs in up to 20% of patients before glucocorticoid therapy is commenced

  • Anterior ischaemic optic neuropathy (AION) owing to arteritis of the posterior ciliary arteries is the most common cause of visual loss in GCA and must be differentiated from non-arteritic AION

  • Cerebrovascular accidents — stroke and transient ischaemic attack — occur in 1.5–7% of patients with GCA and are caused by stenosis or occlusion of the extradural vertebral or carotid arteries

  • A previous ischaemic event in GCA is the strongest predictor for a subsequent event; patients with traditional cardiovascular risk factors and a lower inflammatory response are more likely to develop ischaemic manifestations

  • Adequate doses of glucocorticoids in GCA largely prevent further cranial ischaemic events, but are scarcely effective at improving established visual loss

  • Fast-track clinics for the diagnosis of GCA might substantially reduce the occurrence of permanent sight loss by reducing diagnostic delay.

Abstract

Giant cell arteritis (GCA) is the most common form of vasculitis in individuals aged 50 years and over. GCA typically affects large and medium-sized arteries, with a predilection for the extracranial branches of the carotid artery. Patients with GCA usually present with symptoms and signs that are directly related to the artery that is affected, with or without constitutional manifestations. The most dreaded complication of GCA is visual loss, which affects about one in six patients and is typically caused by arteritis of the ophthalmic branches of the internal carotid artery. 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 twenty years have also contributed to a substantial decline in the frequency of permanent visual loss. 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. The inflammatory response at the onset of arteritis, its role as a predictor of complications and the role of traditional cardiovascular risk factors have been extensively investigated in the past decade. In this Review, the epidemiology, risk factors, clinical presentation and current therapeutic approach of GCA-related ischaemic events are discussed, with a particular emphasis on visual loss.

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Figure 1: Visual loss in a patient with GCA owing to anterior ischaemic optic neuropathy.
Figure 2: Intracranial and extracranial large vessel involvement secondary to giant cell arteritis.

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Acknowledgements

This article is dedicated to Sohan Singh Hayreh, ophthalmologist and clinical scientist, who has been one of the pioneers in the field of vascular diseases of the eye and the optic nerve.

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Authors and Affiliations

  1. Division of Rheumatology, Azienda Ospedaliera, Istituto di Ricovero e Cura a Carattere Scientifico di Reggio Emilia, Viale Risorgimento 80, 42100, Reggio Emilia, Italy

    Alessandra Soriano, Francesco Muratore, Nicolò Pipitone, Luigi Boiardi & Carlo Salvarani

  2. Campus Bio-Medico, University of Rome, Via Álvaro del Portillo 200, Rome, 00128, Italy

    Alessandra Soriano

  3. Università di Modena e Reggio Emilia, Via Università 4, Modena, 41121, Italy

    Francesco Muratore & Carlo Salvarani

  4. Division of Ophthalmology, Azienda Ospedaliera, Istituto di Ricovero e Cura a Carattere Scientifico di Reggio Emilia, Viale Risorgimento 80, 42100, Reggio Emilia, Italy

    Luca Cimino

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Glossary

Fundoscopy

A routine examination (also known as ophthalmoscopy) for looking at the back of the eye (fundus)

Amaurosis fugax

Visual loss in one or both eyes that is transient and painless.

Diplopia

Double vision.

Cortical blindness

Blindness resulting from ischaemia of the visual cortex.

Stenosis

Abnormal narrowing of a blood vessel.

Vasa vasorum

A network of small blood vessels that supply the walls of blood vessels

Cotton wool spots

An abnormal manifestation where fluffy white patches are observed on the retina during fundoscopy examination.

Jaw claudication

Pain in the jaw, particularly when talking or eating

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Soriano, A., Muratore, F., Pipitone, N. et al. Visual loss and other cranial ischaemic complications in giant cell arteritis. Nat Rev Rheumatol 13, 476–484 (2017). https://doi.org/10.1038/nrrheum.2017.98

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