The Birmingham Vasculitis Activity Score (BVAS) is a validated tool for assessment of disease activity in patients with many different forms of vasculitis.
The continuing morbidity of patients with vasculitis, despite the improved prognosis with aggressive therapy, underlines the need for accurate disease assessment.
The BVAS is the most effective validated tool to document disease activity. BVAS v3 consists of a list of items from nine organ systems, which reflect the typical features of active systemic vasculitis. It provides valid definitions for remission and response to therapy as well as flare.
BVAS v3 is valid, reliable and has been widely used in clinical trials in vasculitis to define the responsiveness to various agents including cyclophosphamide, methotrexate, mycophenolate, intravenous immunoglobulin and rituximab.
Whilst the BVAS is used to record current disease activity, a complementary tool called the Vasculitis Damage Index (VDI) can be used to record acculumating damage as a result of vasculitis and/or it’s treatment or other comorbidity occurring after vasculitis was diagnosed, regardless of cause.
The BVAS assess disease activity, flare, remission and the chronic effects of systemic vasculitis. The methods are practical and clinically based to enable standardized measurements of disease status, which consist of measuring:
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The initial iteration of BVAS, known as BVAS version 1, was introduced in 1994. It was a physician-derived scoring system that aimed to quantify the extent and severity of disease manifestations in different organ systems commonly affected by vasculitis. The score encompassed a range of clinical and laboratory parameters, assigning numerical values to various signs and symptoms indicative of disease activity.
Recognizing the dynamic nature of vasculitis and the importance of capturing changes in disease status over time, subsequent versions of BVAS were developed. BVAS version 2, incorporated modifications to enhance its sensitivity and reliability. This version also introduced the concept of weighting items based on their clinical relevance and impact on patient outcomes.
The evolution of BVAS continued with version 3. This current version has further refined the scoring system and included specific criteria for different types of vasculitis, making it more tailored to the diverse manifestations of these disorders. BVAS version 3 also incorporates input from both physicians and patients, reflecting a broader perspective on disease impact.
Details of the development protocols for the BVAS can be found in the Publications section.
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Each item should be recorded as present only if the clinician judges it to be due to active vasculitis and intends to give immunosuppressive therapy or escalate immunosuppressive therapy or escalate careful observation of a patient. This concept underpins the use of BVAS v3 because many items listed in BVAS could be present as result of other conditions (including previously active disease, damage, infection, drug toxicity or comorbidity).
It is essential that the items recorded in BVAS v3 should only be recorded after consideration of the cause. This is semi-subjective because some of the evidence is derived from the patient history and physical examination and cannot always be confirmed with more objective testing.
BVAS v3 provides you with a checklist of potential features that typically occur in active vasculitis and could be present at any time in any patient. If these items are recorded, you should be aware that by recording their presence you are indicating your opinion that the items represent active vasculitis and would therefore be likely to lead to a significant escalation of the patient’s immunosuppressive therapy. Each item is weighted and a maximum score is applied to each system. When the scores from each system are summed, the total score ranges from 0-63 (or 0-33 for persistent disease items), providing an overall quantitative measure of disease activity.
However, the checklist also provides you with a detailed qualitative description of the pattern of disease activity present in any individual patient at any one time.
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The Luqmani Group have developed comprehensive case-study based training modules for the BVAS and VDI and facilitate the training online or in-person to ensure valid use in studies. They have developed a training website to deliver the case studies with automated feedback and have collaborated with academics and industrial partners to ensure quality control in disease evaluation.
For more information, please contact David Gray and Raashid Luqmani via bvasvdi@ndorms.ox.ac.uk.
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