Background/Purpose:
To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS), a validated tool for assessment of vasculitis disease activity, in a cohort of patients with GCA.
Methods:
Patients with GCA enrolled in a prospective, multicenter, longitudinal study were included. All subjects were followed with serial standardized clinical assessments. Symptoms attributable to vasculitis, BVAS, and physician global assessments (PGA) (scale 0-10) were available at each visit. Patients with a BVAS >0 at any visit, or those with symptoms of active vasculitis, were included. Manifestations of active vasculitis captured under the “Other” category of BVAS were scored as 0. Spearman’s rank correlation was used to explore the association of BVAS with other measures of disease activity including PGA, physician rated disease activity (low, moderate or high), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP).
Results:
During a mean (SD) follow-up of 2.3 (1.6) years, symptoms of active GCA were present in 236 visits in 136 subjects (100 female, 74%). Median (range) of BVAS1 (new or worse symptoms) was 1 (0-11), median (range) of BVAS2 (persistent symptoms) was 0 (0-5), and median (range) PGA was 4 (0-9) during the 236 episodes of active disease. In 32 encounters (14%), both BVAS1 and BVAS2 were 0. Clinical manifestations sorted by organ systems on BVAS are displayed in Table 1. Several vascular and ischemic symptoms attributed to active GCA on standardized history forms were captured in the “Other” category and did not contribute to the composite scores on BVAS, including diplopia (4 episodes, 2%), temporal artery tenderness (43 episodes, 18%), carotidynia (16 episodes, 7%), jaw/tongue claudication (65 episodes, 28%), upper extremity claudication (34 episodes, 15%), and lower extremity claudication (12 episodes, 5%).
Table 2 contains correlations of BVAS and other measures of disease activity. BVAS1 correlated with PGA and disease activity while BVAS2 was negatively correlated with both. Neither BVAS subscore correlated with ESR or CRP.
Conclusion:
The BVAS has limited utility in GCA. BVAS1, but not BVAS2, correlates positively with PGA. Several patients in this study with active GCA had a BVAS of 0. Items in 7 of the 9 organ systems on BVAS are not applicable to most patients with active GCA and important symptoms attributable to GCA often fall into the “Other” BVAS section and are not captured in the composite score. Modification of the BVAS with retention of a set of general items and inclusion of large-vessel vasculitis disease-specific items may provide more accurate disease assessment in GCA.
|
Table 1: Frequency of clinical manifestations in 236 encounters of giant cell arteritis with active disease during observation, as captured by the Birmingham Vasculitis Activity Score (BVAS). BVAS items are arranged by organ systems. |
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|
BVAS items (by organ system) |
New/worse symptoms* |
Persistent symptoms* |
|
General (N=193) |
|
|
|
Malaise |
57 |
17 |
|
Myalgia |
51 |
7 |
|
Arthralgia/arthritis |
49 |
17 |
|
Headache |
94 |
9 |
|
Fever (<38.5 degrees Celsius) |
4 |
2 |
|
Fever (>38.5 degrees Celsius) |
2 |
1 |
|
Weight loss (≥2 kilograms) |
24 |
1 |
|
*Median (range) BVAS for category |
1 (0-3) |
0 (0-2) |
|
Cutaneous (N=1) |
|
|
|
Ulcer |
1 |
0 |
|
*Median (range) BVAS for category |
0 (0-4) |
0 (0-0) |
|
Mucous membranes/eyes (N=34) |
|
|
|
Mouth ulcers |
0 |
1 |
|
Blurred vision |
29 |
1 |
|
Sudden vision loss |
12 |
0 |
|
*Median (range) total BVAS for category |
0 (0-6) |
0 (0-2) |
|
Ear, Nose, and Throat (N=1) |
|
|
|
Sinus involvement |
0 |
1 |
|
*Median (range) total BVAS for category |
0 (0-0) |
0 (0-1) |
|
Chest (N=9) |
|
|
|
Persistent cough |
8 |
1 |
|
*Median (range) total BVAS for category |
0 (0-2) |
0 (0-1) |
|
Cardiovascular (N=2) |
|
|
|
Congestive heart failure |
0 |
2 |
|
*Median (range) BVAS for category |
0 (0-0) |
0 (0-2) |
|
Abdominal (N=1) |
|
|
|
Severe abdominal pain |
1 |
0 |
|
*Median (range BVAS for category) |
0 (0-3) |
0 (0-0) |
|
Renal (N=0) |
|
|
|
*Median (range) BVAS for category |
0 (0-0) |
0 (0-0) |
|
Nervous system (N=4) |
|
|
|
Organic confusion/dementia |
2 |
0 |
|
Stroke |
1 |
0 |
|
Sensory peripheral neuropathy |
0 |
1 |
|
*Median (range) BVAS for category |
0 (0-9) |
0 (0-3) |
|
Other N=85 |
63 |
22 |
|
N=total number with any clinical manifestation in that organ system |
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Table 2: Correlations of BVAS with other parameters used to assess disease activity in GCA. |
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|
|
BVAS1 |
p-value |
BVAS2 |
p-value |
PGA |
p-value |
|
PGA |
0.45 |
<0.001 |
-0.20 |
0.002 |
– |
|
|
ESR |
0.02 |
0.78 |
-0.13 |
0.06 |
0.09 |
0.18 |
|
CRP |
0.06 |
0.39 |
-0.02 |
0.73 |
-0.003 |
0.98 |
|
Disease activity* |
0.43 |
<0.001 |
-0.17 |
0.01 |
0.91 |
<0.001 |
|
BVAS= Birmingham Vasculitis Activity Score; BVAS1= score for new/worse disease activity; BVAS2= score for persistent disease activity; GCA= Giant cell arteritis; *rated by evaluating physician as low, medium or high. |
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Disclosure:
T. A. Kermani,
None;
D. Cuthbertson,
None;
S. Carette,
None;
G. S. Hoffman,
None;
N. A. Khalidi,
None;
C. L. Koening,
None;
C. A. Langford,
None;
K. McKinnon-Maksimowicz,
None;
C. McAlear,
None;
P. A. Monach,
Genentech and Biogen IDEC Inc.,
2;
P. Seo,
None;
K. J. Warrington,
None;
S. R. Ytterberg,
None;
P. A. Merkel,
None;
E. L. Matteson,
None.
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