A new era for giant cell arteritis

Abstract

The landscape of the investigation and management of giant cell arteritis (GCA) is advancing. In this review we will outline the recent advances by searching the current English literature for relevant articles using key words of giant cell arteritis, temporal arteritis, Horton's disease, investigation, and treatment. Delay in diagnosis, diagnostic uncertainty and glucocorticoid (GC) morbidity are among the highest concerns of clinicians and patients in this disease area. The positive news is that fast track pathways, imaging techniques and new therapies are emerging for routine management of GCA. Future directions for intervention in the treatment paradigm will be discussed.

Fig. 1

Prominent, beaded, pulseless, tender temporal artery in a patient with biopsy proven giant cell arteritis. Abnormality detected by patient 3 weeks prior to new-onset temporary, then permanent double vision, followed by anterior ischaemic optic neuropathy and complete loss of vision to perception of light

Fig. 2

Diagram of frequency of symptoms of GCA and the American College of Rheumatology (ACR) 1990 criteria for the definite diagnosis of GCA [30, 100, 101]

Fig. 3

These are Temporal artery ultrasound images showing (a) longitudinal and (b) cross sectional images of a normal artery; (c) longitudinal and (d) cross section image of the non-compressible, hypoechoic “halo sign” (white asterisks)

Fig. 4

A 67-year-old Caucasian lady presented with headache, PMR, drenching night sweats, weight loss and CRP 120. ¹⁸FDGPET-CT fused images show ¹⁸FDG avid large-vessel GCA (white arrows) of the thoracic aorta in sagittal (a), coronal (b) and transverse view (c). Inflammatory activity extends into the subclavian and common carotid arteries (d)

Fig. 5

Key histopathological features of GCA. a H&E ×100, superficial temporal artery with marked intimal thickening. b Thick arrow head denotes inflammatory cells in the media and adventitia

Fig. 6

Key histopathological feature of GCA with EHVG stain ×200 thick arrow head points to fragments of internal elastic laminae remaining and duplication

Fig. 7

Key histopathological feature of healed arteritis. a Thick arrow head shows calcification at the intima media border and b an elastic stain (EHVG) highlights significant disruption of the internal elastic lamina with defects extending over 30–50% of the circumference of the artery

Fig. 8

Percentage of new co-morbidities or adverse events recorded following a diagnosis of GCA cohort as adpated from Proven et al. [71]