Birmingham Vasculitis
Activity Score for
Wegener’s Granulomatosis (BVAS/WG)
An Instructions Manual
Purpose of assessment
BVAS/WG is designed to document clinical features
that are directly due to active WG. In addition, the instrument separates the
features that represent new or worse disease activity from those that represent Persistent
activity. In scoring BVAS/WG, it is very important not to confuse activity with damage.
Damage, defined as the presence of non-healing scars, and is a concept distinct from
current disease activity. Damage is scored separately in another index, the Vasculitis
Damage Index, which is not the subject of this exercise.
Recording disease activity
The list of items in BVAS/WG includes clinical
symptoms and signs, as well as information obtained from additional tests (e.g., chest
x-rays) or subspecialty consultations. When using the BVAS/WG evaluation form, one scores
only these items attributable to currently active WG (after the exclusion of obvious
causes such as infection, hypertension, and treatment toxicity). BVAS/WG scores may vary
rapidly, and reflect the need for therapy.
New Patients
If the patient is being evaluated for the first
time and has not been treated, all of the abnormalities noted should be recorded as
NEW/WORSE (m ) regardless of their duration. After going through the entire items
list, remember to consider adding any other significant items to the “Other”
section, if relevant. A list of “Other” items that might be included in these
sections is displayed at the end of this Manual. If a section has no items present, check
the “none” box.
Follow-up Patients
If the patient is being evaluated in follow-up,
there may be some abnormalities that are NEW or WORSE (m) within the previous 28
days. Other abnormalities may have been present on the previous assessment and are neither
new nor worse, but rather still present (PERSISTENT p ). By making this distinction, one
differentiates new, acute disease activity from Persistent disease activity. It is
important to remember that Persistent activity is activity, not damage.
Thus, Persistent purpura should be scored as activity. In contrast, weakness from
mononeuritis multiplex of 4 months duration is damage, and should not be scored in
BVAS/WG. Sometimes it may be difficult to be certain whether a symptom or sign is
due to Persistent activity or to damage. As in caring for real patients, in evaluating
such cases one relies on clinical judgement to make this distinction.
Checking the Boxes
Check one of the boxes for each item (m or p) only if you ascribe the abnormality to the presence of active WG. If no abnormalities
ascribable to WG are present in a given organ system, check the “none” box. In
this way, we can be certain that you did not overlook an organ system on the scoring
sheet. Sometimes you will have patients in whom abnormalities are present that are not due
to WG (e.g., hematuria due to urinary infection or cyclophosphamide toxicity). In these
cases, you should NOT record them in the BVAS/WG list, even though they are present,
because they are not caused by active WG. In some patients, abnormalities that were due to
previous episodes of WG may still be evident, even though the disease is entirely inactive
(e.g., stroke). These features should also NOT be recorded on BVAS/WG, since they
represent non-healing scars (damage).
m Check this box only if the abnormality is
NEW/WORSE within the previous 28 days (unless this is the first presentation
of untreated disease).
p Check this box only if the abnormality is PERSISTENT since the last assessment
and not worse within the previous 28 days.
Check the “None” box if there is not a single Major or Minor item that is New/Worse within a particular organ system.
Necessity for “Judgement Calls”
As in clinical practice, one must sometimes make
“judgement calls” in scoring BVAS/WG. For example, Persistent sinus symptoms may
be difficult to classify with certainty as either active disease or permanent damage.
Similarly, small amounts of hematuria (usually with RBC casts) may persist for months in
patients whose disease is otherwise quiescent. In both such cases, the physician is
unlikely to intensify treatment in the absence of other indications of active disease. For
this reason, these findings (and analogous findings in other organ systems) should not be
scored in BVAS/WG. If subsequent events cause you to re-consider your judgement call, you
may amend your original scoring.
You will note that for some features (e.g., sensorineural deafness, most of the neurological items) there is no Persistent box. This reflects the fact that if these features are present, they are New/Worse by definition. If the feature is still present at subsequent assessments – as mononeuritis multiplex is likely to be – the feature is (by definition) damage, and should not be scored.
Recording Major and Minor Items
Individual items are defined as Major by the
presence of an asterisk (*). All other items are defined as Minor. If you list additional
items in the “Other” section, you should indicate whether the item is
“Major” or “Minor.” In general, a Major item is one whose presence
would prompt the use of cyclophosphamide. Minor items are those more likely to be
treated with methotrexate or an increase in prednisone.
If you decide that a particular abnormality is due to the presence of active WG, you must distinguish problems that are New/Worse from those problems that are Persistent. For each item where there is an abnormality, you need to check either the NEW/WORSE box or the PERSISTENT box, but not both.
Calculating the BVAS/WG Score
BVAS/WG is calculated by adding all of the Major
(*) items marked in the New/Worse column, and entering the sum in the appropriate box on
the right side of the page. This is repeated for the Minor items in the NEW/WORSE column,
and then do the same for the Major and Minor items in the Persistent column.
Defining disease status
We propose the following definitions of status,
based on evaluation of the BVAS/WG:
Severe disease/flare: If any Major item is recorded, the patient has a “Severe Flare.”
Limited disease/flare: If any Minor item is recorded, the patient has a “Limited Flare.”
Persistent disease: Persistent disease indicates the presence of 1 or more Persistent items attributed to active disease.
Remission: Remission indicates no active disease (i.e., no New/Worse and no Persistent items present).
Physician’s Global Assessment
Finally, use the 10 cm horizontal line to record
your assessment of the overall disease activity in this case. Remember that you should not
be influenced by the presence of any accumulated damage, complication of treatment,
social/emotional problems, or other issues not related to active WG.
The Cleveland Clinic | Contact Us | October 26, 2000
