Presenting features and outcomes of cranial-limited and large-vessel giant cell arteritis: a retrospective cohort study

Abstract

Objectives: To compare the presenting features and outcomes of patients with cranial-limited (C-) and large-vessel (LV-) giant cell arteritis (GCA).

Methods: Data from our GCA cohort were collected retrospectively. Patients who underwent total-body large-vessel imaging within 10 days after commencing steroid therapy were included. Patients with LV involvement were classified as LV-GCA. Presenting features, treatments, and outcomes of LV-GCA and C-GCA patients were compared.

Results: 161 patients were included (LV-GCA, n = 100). At baseline, LV-GCA patients were younger than those with C-GCA (73.2 ± 8.9 vs 76 ± 8.8 years, p = 0.018) and had a longer delay to diagnosis (3.5 ± 4.6 vs 2.3 ± 4.9 months, p = 0.001). C-GCA patients had a higher incidence of headache (p = 0.006) and ischaemic optic neuropathy (p < 0.001), whereas LV-GCA patients had more systemic symptoms (fever, p = 0.002; fatigue, p < 0.001; weight loss, p < 0.001; night sweats, p = 0.015) and dry cough (p = 0.031). Corrected cumulative prednisone dose, relapse-free survival, relapse-rate, and incidence of ascending aortic aneurysms were not significantly different between the two subgroups. A steroid-sparing agent was added in 73% of LV- and 55.7% of C-GCA patients (p = 0.027), but was introduced more frequently at baseline in LV-GCA patients (52% vs 23.5%, p = 0.006). LV-GCA patients initially treated with glucocorticoid monotherapy relapsed sooner (relapse-free survival, HR = 0.56, 95% CI 0.41–0.78, p < 0.001) and had a higher relapse rate (relapses per 10 person-years, 6.73 ± 11.50 vs 3.82 ± 10.83, p = 0.011).

Conclusion: LV-GCA patients were younger at diagnosis and suffered a longer diagnostic delay. The outcomes of the two subgroups were similar. An earlier introduction of steroid-sparing agents in LV-GCA patients might have played a positive role.

Acknowledgements

The authors thank Dr. Jamie King for language revision of the paper.

Disclosure statement

No potential conflict of interest was reported by the authors.

Supporting information

Additional supporting information may be found in the online version of this article.

Supplementary table S1. Clinical features of the eight giant cell arteritis patients with large-vessel involvement and a positive temporal artery biopsy. Supplementary table S2. Incidence of ascending aortic aneurysm at 24 and 60 months in patients with giant cell arteritis. Supplementary table S3. Baseline demographic and clinical features of patients with large-vessel giant cell arteritis who received a disease-modifying antirheumatic drug at baseline (DMARD+) and those who were initially treated with glucocorticoids alone (DMARD-). Please note that the editors are not responsible for the content or functionality of any supplementary material supplied by the authors. Any queries should be directed to the corresponding author.