Background Giant cell arteritis (GCA) is the most common systemic vasculitis in patients aged 50 years or older. The presence of cranial features and an abnormal temporal artery biopsy have historically been the primary focus for diagnosis of this condition. A notable percentage of patients with symptoms of GCA have negative temporal artery biopsies, however, few cohort studies exist comparing the presentation and outcome of patients based on temporal artery positivity.
Objectives To establish a large, single institution cohort of patients with temporal artery biopsy-negative GCA. To identify differences in presentation and outcome among patients with temporal artery biopsy-negative and temporal artery biopsy-positive GCA.
Methods Patients with temporal artery biopsy-negative GCA diagnosed between 1/1/1998 and 12/31/2013 were identified retrospectively. Final diagnosis was confirmed by consensus among two rheumatologists and a physician abstractor. Baseline characteristics and outcomes were compared to a previously established biopsy-positive GCA cohort (n=286) from the same institution.
Results 110 patients with temporal artery biopsy-negative GCA were identified. Unilateral biopsies were performed in 73, bilateral-sequential in 10, and bilateral same day in 27 cases. Median duration between steroid initiation and biopsy was 3 days. Median length of first biopsy was 14mm and second biopsy (if performed) was 22mm. Among biopsy-negative patients with advanced imaging within 6-months of diagnosis, 67% (41/61) had evidence of large vessel vasculitis.
Patients with biopsy-negative GCA were younger (72.0±9.0 vs 75.0±7.6; p=0.001), met fewer ACR criteria (≥3 criteria 64% vs 95%; p<0.001) and had a shorter time from symptom onset to diagnosis (median 1.1 vs 2.1 months; p<0.001). Vascular risk factors evaluated at diagnosis showed a higher rate of pre-existing hypertension and obesity among patient with biopsy-negative GCA but similar rates of smoking and diabetes mellitus. Frequency of headache and vision loss at time of presentation were similar between groups. However, biopsy-negative GCA patients had more temporal artery tenderness (35% vs 16%; p<0.001) and arm claudication (13% vs 2%; p<0.001) but less frequent jaw claudication (19% vs 52%; p<0.001). Anorexia, fatigue, and arthralgia were also more commonly noted in biopsy-negative patients. Baseline CRP was lower among patients with negative biopsies (44.3±53.6 vs 70.4±63.9 mg/L; p<0.001).
Initial prednisone dose was similar among both cohorts. Although cumulative glucocorticoid (GC) was lower in biopsy-negative patients at 1 year (6.3±2.6 vs 7.2±2.7 g; p=0.004), cumulative GC doses at 2-years and 5-years were equivalent. Biopsy-positive patients (5-years, 56±3%) were able to discontinue GC sooner than biopsy-negative patients (5-years, 30±5%; p<0.001). The number of relapses, time-to-first relapse, annual relapse rate and mortality did not differ based on biopsy positivity.
Conclusions While similarities are present, notable differences are observed at diagnosis in patients with biopsy-negative GCA. Further research is needed to confirm and understand the variability in GC duration.
Disclosure of Interest None declared
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