Performance of the birmingham vasculitis activity score and disease extent index in childhood vasculitides

Erkan Demirkaya, Seza Ozen, Angela Pistorio, Roberta Galasso, Angelo Ravelli, Rachana Hasija, Esra Baskin, Frank Dressler, Michel Fischbach, Julia Garcia Consuegra, Nicolae Iagaru, Srdjan Pasic, Salvatore Scarpato, Marion A J van Rossum, Maria Teresa Apaz, Judith Barash, Giuseppina Calcagno, Benito Gonzalez, Esther Hoppenreijs, Maka IoselianiHenryka Mazur-Zielinska, Olga Vougiouka, Nico Wulffraat, Raashid Luqmani, Alberto Martini, Nicolino Ruperto, Pavla Dolezalova

Research output: Contribution to journalArticlepeer-review


Objectives: 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. Conclusion: 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.

Original languageEnglish
JournalClinical and Experimental Rheumatology
Issue numberSUPPL. 70
Publication statusPublished - 2012


  • Birmingham vasculitis activity score
  • Childhood vasculitis
  • Disease activity assessment
  • Disease extent index
  • Outcome measurement

ASJC Scopus subject areas

  • Rheumatology
  • Immunology
  • Immunology and Allergy


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