Background/Purpose: A subset of patients with giant cell arteritis (GCA) has large vessel (LV) involvement. We sought to identify baseline variables that distinguish patients with GCA with subclavian artery involvement (LV-GCA) from those with cranial disease (C-GCA), and to compare treatment, long term follow-up and prognosis of these two subtypes of GCA.
Methods: All patients over age 50 years with radiographic evidence of subclavian LV-GCA diagnosed between January 1, 1999 and December 31, 2008 were identified in this retrospective study. These were compared to a cohort of patients with biopsy-positive C-GCA diagnosed in the same period.
Results:
The study included 120 patients with radiographic evidence of LV-GCA and 212 patients with C-GCA. LV-GCA was noted at GCA diagnosis in 90 patients, and after GCA in 30 patients (mean time 2.6 years from diagnosis of GCA). Temporal artery biopsy (TAB) was performed in 79 patients with LV-GCA, and was positive in 41 (52%). In addition to subclavian vasculitis, 56% of LV-GCA cases had radiographic evidence of thoracic aortic involvement (thickening or aneurysm/ectasia).
Compared to patients with C-GCA, patients with LV-GCA were younger (mean age 68.2 + 7.5 years vs 75.7 + 7.4, p <0.001), more likely to have a prior history of PMR (26% vs 15%, p = 0.01) and had longer duration of symptoms at GCA diagnosis (median 3.5 months vs 2.2 months p <0.001). Cranial symptoms (41% vs 83%, p <0.001) and vision loss (4% vs 11%, p = 0.04) were less frequent in LV-GCA compared with C-GCA. Upper extremity claudication (52% vs 0%, p <0.001), Raynaud’s phenomenon (11% vs 0%, p <0.001), vascular bruits (38% vs 9%, p <0.001) and abnormal pulse exam (60% vs 14%, p <0.001) were more frequent in LV-GCA compared to C-GCA. There were no differences in cardiovascular risk factors, sex distribution and ESR/CRP between the 2 cohorts. ACR classification criteria for GCA were satisfied in 39.2% of LV-GCA and in 95.3% of C-GCA patients (p <0.001). Patients in the C-GCA cohort met a median of 4.0 ACR criteria for GCA, while the LV-GCA patients met only a median of 2.0 criteria (p <0.001).
103 patients with LV-GCA and 167 patients with C-GCA had a follow-up period longer than 6 months (median follow up 3.6 and 4.6 years respectively). Relapse rate was significantly higher (48.6 vs 29.8/100 person-yrs, p <0.001) and time to first relapse was shorter (median 0.8 vs 1.2 yrs, p = 0.005) for LV-GCA compared with C-GCA. The cumulative corticosteroid (CS) dose at 1 year was higher (11.4 + 5.9 grms vs 9.1 + 3.7, p <0.001) and the time to discontinuation of CS therapy was longer (median 4.5 vs 2.2 yrs, p <0.001) in LV-GCA. At 5 years of follow-up, the rate of incident aortic aneurysm was higher in LV-GCA (1.5% vs 0.3%, p = 0.005) compared with C-GCA. Rate of incident stroke was similar in the 2 GCA cohorts.
Conclusion:
In this large study, patients with LV-GCA presented less often with cranial symptoms and more often with findings of large-vessel insufficiency. Although patients with LV-GCA had a lower rate of vision loss, they had a higher relapse rate, greater CS requirements and an increased incidence of aortic aneurysm compared with C-GCA. The ACR criteria for GCA are inadequate for the classification of patients with LV-GCA.
Disclosure:
F. Muratore,
None;
T. A. Kermani,
None;
C. S. Crowson,
None;
A. B. Green,
None;
E. L. Matteson,
Centocor, Inc./Johnson and Johnson,
2,
Genentech and Biogen IDEC Inc.,
2,
Hoffmann-La Roche, Inc.,
2,
Human Genome Sciences, Inc.,
2,
Pfizer Inc,
2,
Novartis Pharmaceutical Corporation,
2,
Roche Pharmaceuticals,
2,
UCB Group,
2,
Centocor, Inc.,
5,
Horizon Pharma,
5,
Novartis Pharmaceutical Corporation,
5;
K. J. Warrington,
None.
ACR Meeting Abstracts - https://acrabstracts.org/abstract/large-vessel-giant-cell-arteritis-a-cohort-study/