Background/Purpose: Giant cell arteritis (GCA) involves both the cranial and large vessels. Studies have shown that while the vision loss rates are higher, the relapse rates may be lower in patients with cranial arteritis without large vessel involvement (pure cranial arteritis). However, in many studies, the definition of pure cranial arteritis has not been accurate, as the supraaortic tree has not been thoroughly examined and they are based mainly on retrospectively collected data. This study aimed to investigate the rates of vision loss and relapse in patients with pure cranial GCA in a Norwegian prospective cohort of GCA patients.

Methods: Patients with new-onset GCA referred to the Department of Rheumatology, Martina Hansens Hospital in Bærum, Norway are prospectively included and followed-up. The diagnosis is based on typical for GCA clinical manifestations and ultrasound findings. All the patients are scanned by ultrasound using the anteromedial approach for the supraaortic vessels (carotid, vertebral, subclavian, axillary proximally, and distally) and cranial vessels (temporal, facial) as well. The examination utilizes a General Electric S8 ultrasound machine with a 9-12 MHz linear probe for the large vessels and an 18 MHz hockey stick probe for the cranial arteries and a Canon Aplio 700 with an 11 MHZ  and 22 MHz probes for the large and cranial vessels respectively. The age, gender, CRP, Prednisolone dose, distribution of vasculitis in the vessels, vision loss, and relapse rates are recorded and relative risks (RR) are calculated.

Results: Seventy-nine patients, 58 (73%) females, and 21 (27%) males were diagnosed with GCA until May 2020. The mean age was 72 years.  Mean CRP was 96 mg/dl (95% CI (63-96)). Of the 79 GCA patients, 20 had involvement of the cranial vessels only (pure cranial CGA), 19 patients have involvement of the large supraaortic vessels only, and 50 of both cranial and large supraaortic vessels. Seven patients suffered from vision loss (9%) and all these patients had a concomitant cranial disease. The GCA patients with pure cranial disease had a RR for visual loss of 1.14 (95% CI (0.3-3.9)) while GCA patients with both cranial and large vessel disease had a RR of 1.35 (95% CI (1.2-1.5)). No patients with pure large vessel GCA suffered from vision loss. The median Prednisolone dose at the first relapse was 10 mg (IQR 8). Of the 20 GCA patients with cranial arteritis, only 3 (4%) relapsed during the study period, while 33 (25 %) patients of the 59 GCA patients with concomitant large supraaortic vessels involvement relapsed. The Relative Risk for relapse in patients with pure cranial arteritis was 0.3 (95%CI 0.1-0.8).

Conclusion: In a prospective cohort of GCA patients, pure cranial disease appears to have a 14 % higher risk of vision loss and a 70% lower risk of relapse. It seems that concomitant large supraaortic vessel involvement does not reduce the risk of vision loss. We continue to recruit GCA patients in our prospective cohort and examine the RR of the vision loss and relapse in larger numbers of patients.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/risk-for-vision-loss-and-relapse-in-patients-with-giant-cell-arteritis/