Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Validation of Birmingham Vasculitis Activity Score in Childhood Vasculitis.

Demirkaya1,  Erkan, Ozen3,  Seza, Pistorio3,  Angela, Ravelli3,  Angelo, Hasija3,  Rachana, Khubchandani3,  Raju, Scarpato3,  Salvatore

IRCCS G. Gaslini, Genova, Italy
IRCCS G. Gaslini/Università degli Studi di Genova


Unlike for adults patients with systemic vasculitis, there are no validated tools to measure disease activity status in childhood (c-) systemic vasculitis.


Our aim was to validate the last version of Birmingham Vasculitis Activity Score (BVAS v.3) and the Disease Extend Index (DEI) for comprehensive assessment of paediatric primary c-systemic vasculitis.


We extracted from the PRINTO database all patients who fulfilled the Henoch-Schoenlein (HSP), childhood (c) polyarteritis nodosa (c-PAN), c-Wegener (c-WG) and c-Takayasu (c-TA) EULAR/PRINTO/PRES c-vasculitis classification criteria and whose disease duration at the time of diagnosis was <= 3 months. Data were also available for follow-up evaluation >= 3 months after diagnosis. The validation of the BVAS and DEI were examined by assessing discriminant ability among the 4 vasculitis, convergent validity by Spearman correlation coefficient with physician's global assessment of disease activity (MD global), indexes of inflammation (ESR/CRP), responsiveness to change over time through the standardizes response mean (SRM); A SRM value <0.5 is considered small, >=0.5 and <0–8 moderate, and values >= 0.8 represent large effect.


The analysis data set included 796/1124 (71%) patients (M:F 0.96:1): there were 669 HSP, 80 c-PAN, 25 c-WG and 22 c-TA. The median age of the diagnosis was 6.99-year (6.6–11.96) and median delay for the diagnosis from the onset of signs or symptoms was 0.01 (0.003–0.027) years. In the table 1 are reported the 9 subscore of the BVAS, the total BVAS score, the DEI, the MD global and indexes of inflammation.

The BVAS was able to discriminate between the 4 c-vasculitides with total BVAS scores equal to 9 (6.0–14.0), 17.5 (11.5–24.5), 23 (19–29), 15 (11–20) in HSP, c-PAN, c-WEG and c-TA respectively.

Table 1. Discriminant ability of the BVAS total scores and the other tools:data are medians (1*-3* quartiles)

 All patientsn = 796HSPn = 669 (%)c-PANn = 80 (%)c-WGn = 25 (%)c-TAn = 22 (%)P values*
BVAS10 (8.0–16.0)9 (6.0–14.0)17.5 (11.5–24.5)23 (19–29)15 (11–20)<0.001
DEI5 (4.0–6.0)5 (4.0–6.0)5 (3.0–7.0)7 (6–9)3 (2–5)<0.001
MD global5 (3–7)4 (2.5–6)7 (7–8)8.5 (8–9)8 (7–8.5)<0.001
CRP1.38 (0.5–4.33)1.01 (0.45–2.21)8.14 (4.45–16.4)9.8 (5.36–16.38)4.63 (2.23–8.49)<0.001
ESR29 (14.0–51.5)22 (12.0–36.5)86 (53.0–115.0)76.5 (58–107.5)48 (40–72)<0.001
*P values refers to ANOVA with Dunn test as a posthoc.

The cutaneous and cardiovascular sub-score are able to distinguish TA patients from the others; the ENT sub-score shows a significant ability to discriminate the c-WG patients from the others.

A strong correlation was found between the BVAS and DEI (rs=0.80) while correlation with MD global were moderate (rs=0.49) and poor with CRP and ESR (rs=0.34, rs=0.31). Responsiveness was large for BVAS total score (SRM=1.38), DEI (SRM=1.9), MD global (SRM=1.3) and ESR (SRM=0.85).


BVAS and DEI showed adequate discriminant ability and sensitivity to change but, poor to moderate convergent validity. Further work is needed in order to improve activity measurement in c-vasculitides.

To cite this abstract, please use the following information:
Demirkaya, Erkan, Ozen, Seza, Pistorio, Angela, Ravelli, Angelo, Hasija, Rachana, Khubchandani, Raju, et al; Validation of Birmingham Vasculitis Activity Score in Childhood Vasculitis. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :1705
DOI: 10.1002/art.29470

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