Performance of Birmingham Vasculitis Activity Score and disease extent index in childhood vasculitides.
OBJECTIVE
To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) v3 and the Disease Extent Index (DEI) for the assessment of disease activity in 4 primary childhood (c-) systemic vasculitides.


METHODS
Patients fulfilling the EULAR/PRINTO/PRES (Ankara) c-vasculitis classification criteria for Henoch-Schönlein purpura (HSP), childhood (c) polyarteritis nodosa (c-PAN), c-Wegener's granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) with disease duration at the time of diagnosis ≤3 months were extracted from the PRINTO database. The performance of the BVAS and DEI were examined by assessing convergent validity, the pattern of disease involvement, and responsiveness. We also evaluated alternative unweighted scoring methods for both tools.


RESULTS
The analysis set included 796 patients with 669 HSP, 80 c-PAN, 25 c-WG and 22 c-TA. The median age at diagnosis was 6.9 years (6.6-12) and median delay in making the diagnosis from the onset of signs/symptoms was 0.01 (0.003-0.027) years. A strong correlation was found between the BVAS and DEI (rs=0.78) while correlation with the physician global assessment was moderate (rs=0.48) with BVAS and poor with DEI (rs=0.25). Both the BVAS and DEI sub-scores and total scores were able to descrive the disease involvement in the 4 childhood vasculitides. Responsiveness was large (>1.5) for both tools. The performance characteristics of the BVAS and DEI with the unweighted methods were comparable.


CONCLUSIONS
This study demonstrates that both the BVAS and DEI are valid tools for the assessment of the level of disease activity in a large cohort of childhood acute and chronic vasculitides.
IRCCS G. Gaslini, Pediatria II, Reumatologia, PRINTO, Genova, Italy. dottore_erkan@yahoo.com
Erkan
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Demirkaya
Seza
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Ozen
Angela
A
Pistorio
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R
Galasso
Angelo
A
Ravelli
Rachana
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Hasija
Esra
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Baskin
Frank
F
Dressler
Michel
M
Fischbach
Julia
J
Garcìa Consuegra
Nicolae
N
Iagaru
Srdjan
S
Pasic
Salvatore
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Scarpato
Marion A J
MA
van Rossum
Maria Teresa
MT
Apaz
Judith
J
Barash
Giuseppina
G
Calcagno
Benito
B
Gonzalez
Esther
E
Hoppenreijs
Maka
M
Ioseliani
Henryka
H
Mazur-Zielinska
Olga
O
Vougiouka
Nico
N
Wulffraat
Raashid
R
Luqmani
Alberto
A
Martini
Nicolino
N
Ruperto
Pavla
P
Dolezalova
D002648:Child; D003937:Diagnosis, Differential; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis; D006305:Health Status Indicators; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis; D010488:Polyarteritis Nodosa / Q000175:diagnosis; D011237:Predictive Value of Tests; D011379:Prognosis; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D013625:Takayasu Arteritis / Q000175:diagnosis; D009626:Terminology as Topic; D014657:Vasculitis / Q000145:classification / Q000175:diagnosis*