Birmingham Vasculitis Activity Score (BVAS) v3

BVAS is a comprehensive multisystem clinical assessment used in therapeutic studies of systemic vasculitis.

Refer to the text below the calculator for more information on the BVAS v3 and its administration.


The BVAS v3 assesses symptoms and signs of systemic vasculitis, both persistent and new or worsening ones. It consists of 9 sections, each with several items that need to be rated, if present.

Several instructions on how to administer the score and detailed descriptions of the items can be found in the text below.


This is the 3rd version of the Birmingham Vasculitis Activity Score, the most up to date, modified and validated in a 2008 study.

The score consists of 56 items grouped under 9 sections, to provide assessment of systemic vasculitis symptoms.

Persistent scores can range from 0 to 33 whilst the New/Worse scores can range from 0 to 63.


1General

Myalgia
Arthralgia or arthritis
Fever ≥38° C
Weight Loss ≥2 kg

2Cutaneous

Infarct
Purpura
Ulcer
Gangrene
Other skin vasculitis

3Mucous Membranes / eyes

Mouth ulcers / granulomata
Genital ulcers
Adnexal inflammation
Significant proptosis
Scleritis / Episcleritis
Conjunctivitis / Blepharitis / Keratitis
Blurred vision
Sudden visual loss
Uveitis
Retinal changes (vasculitis, thrombosis / exudate / haemorrhage)

4ENT

Bloody nasal discharge / crusts / ulcers / granulomata
Paranasal sinus involvement
Subglottic stenosis
Conductive hearing loss
Sensorineural hearing loss

5Chest

Wheeze
Nodules or cavities
Pleural effusion / pleurisy
Infiltrate
Endobronchial involvement
Massive haemoptysis / alveolar haemorrhage
Respiratory failure

6Cardiovascular

Loss of pulses
Valvular heart disease
Pericarditis
Ischaemic cardiac pain
Cardiomyopathy
Congestive cardiac failure

7Abdominal

Peritonitis
Bloody diarrhoea
Ischaemic abdominal pain

8Renal

Hypertension
Proteinuria
Haematuria
Serum creatinine 125-249 μmol/L
Serum creatinine 250-499 μmol/L
Serum creatinine ≥500 μmol/L
>30% rise in creatinine or >25% fall in creatinine clearance

9Nervous system

Headache
Meningitis
Organic confusion
Seizures (not hypertensive)
Stroke
Spinal cord lesion
Cranial nerve palsy
Sensory peripheral neuropathy
Mononeuritis multiplex
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About the BVAS v3

Version 3 of the Birmingham Vasculitis Activity Score (BVAS) helps clinicians assess persistent and new or worse manifestations in patients with system vasculitis.

The table below introduces the 9 sections (and describes the 56 items) of the score, the maximum points achievable for each section and the points awarded to each item, if present:

Manifestation & Definition Persistent points New/Worse points
1. General Max persistent 2 Max New/Worse 3
Myalgia
Pain in the muscles
1 1
Arthralgia or arthritis
Pain in the joints or joint inflammation
1 1
Fever ≥38° C
Documented oral / axillary temperature. If rectal temperature is measured, raise threshold to 38.5° C
2 2
Weight Loss ≥2 kg
Loss of dry body weight without dieting
2 2
2. Cutaneous Max persistent 3 Max New/Worse 6
Infarct
Area of tissue necrosis or splinter haemorrhages
1 2
Purpura
Subcutaneous or submucosal haemorrhage in the absence of trauma
1 2
Ulcer
A disruption in the continuity of the skin
1 4
Gangrene
Extensive tissue necrosis
2 6
Other skin vasculitis
Livedo reticularis, subcutaneous nodules, erythema nodosum, etc
1 2
3. Mucous Membranes / eyes Max persistent 3 Max New/Worse 6
Mouth ulcers / granulomata
Aphthous stomatitis, deep ulcers, strawberry gingival hyperplasia
1 2
Genital ulcers
Ulcers on the genitalia or perineum
1 1
Adnexal inflammation
Salivary or lacrimal gland inflammation.
2 4
Significant proptosis
>2 mm protrusion of the eyeball
2 4
Scleritis / Episcleritis
Episcleritis Inflammation of the sclera
1 2
Conjunctivitis / Blepharitis / Keratitis
Inflammation of the conjunctiva, eyelids or cornea - but not due to sicca syndrome
1 1
Blurred vision
Deterioration of visual acuity from previous or baseline
2 3
Sudden visual loss
Acute loss of vision
n/a 6
Uveitis
Inflammation of the uvea (iris, ciliary body, choroid)
2 6
Retinal changes (vasculitis, thrombosis / exudate / haemorrhage
Sheathing of retinal vessels or evidence of retinal vasculitis on fluorescein angiography; thrombotic retinal arterial or venous occlusion; soft retinal exudate (exclude hard exudates) / retinal haemorrhage
2 6
4. ENT Max persistent 3 Max New/Worse 6
Bloody nasal discharge / crusts / ulcers / granulomata
Bloody, mucopurulent, nasal secretion, light or dark brown crusts frequently obstructing the nose, nasal ulcers or granulomatous lesions observed on rhinoscopy
2 4
Paranasal sinus involvement
Tenderness or pain over paranasal sinuses (usually confirmed by imaging)
1 2
Subglottic stenosis
Stridor or hoarseness due to inflammation and narrowing of the subglottic area observed by laryngoscopy
3 6
Conductive hearing loss
Hearing loss due to middle ear involvement (usually confirmed by audiometry)
1 3
Sensorineural hearing loss
Hearing loss due to auditory nerve or cochlear damage (usually confirmed by audiometry)
2 6
5. Chest Max persistent 3 Max New/Worse 6
Wheeze
Wheeze on clinical examination
1 2
Nodules or cavities
New lesions detected on imaging
n/a 3
Pleural effusion / pleurisy
Pleural pain and/or friction rub on clinical assessment; radiologically confirmed pleural effusion.
2 4
Infiltrate
Detected on chest X-ray or CT scan
2 4
Endobronchial involvement
Endobronchial pseudotumor or ulcerative lesions. NB: smooth stenotic lesions to be included in VDI; subglottic lesions to be recorded in the ENT section.
2 4
Massive haemoptysis / alveolar haemorrhage
Major pulmonary bleeding, with shifting pulmonary infiltrates
4 6
Respiratory failure
The need for artificial ventilation
4 6
6. Cardiovascular Max persistent 3 Max New/Worse 6
Loss of pulses
Clinical absence of peripheral arterial pulsation in any limb
1 4
Valvular heart disease
Clinical or echo detection of aortic / mitral / pulmonary valve involvement
2 4
Pericarditis
Pericardial pain / friction rub on clinical assessment
1 3
Ischaemic cardiac pain
Typical clinical history of cardiac pain leading to myocardial infarction or angina
2 4
Cardiomyopathy
Significant impairment of cardiac function due to poor ventricular wall motion confirmed on echocardiography
3 6
Congestive cardiac failure
Heart failure by history or clinical examination
3 6
7. Abdominal Max persistent 4 Max New/Worse 9
Peritonitis
Typical abdominal pain suggestive of peritoneal involvement
3 9
Bloody diarrhoea
Of recent onset
3 9
Ischaemic abdominal pain
Typical abdominal pain suggestive of bowel ischaemia, confirmed by imaging or surgery
2 6
8. Renal Max persistent 6 Max New/Worse 12
Hypertension
Diastolic >95 mm Hg
1 4
Proteinuria
>1+ on urinalysis or >0.2g/24 hours
2 4
Haematuria
‘Moderate’ on urinalysis or ≥10 RBC per high power field, usually accompanied by red cell casts
3 6
Serum creatinine 125-249 μmol/L
At first assessment only
2 4
Serum creatinine 250-499 μmol/L
At first assessment only
3 6
Serum creatinine ≥500 μmol/L
At first assessment only
4 8
>30% rise in creatinine or >25% fall in creatinine clearance
Progressive worsening of renal function. Can be used at each assessment if the renal function has deteriorated from prior value
n/a 6
9. Nervous system Max persistent 6 Max New/Worse 9
Headache
Unaccustomed & persistent headache
1 1
Meningitis
Clinical evidence of meningism
1 3
Organic confusion
Impaired orientation, memory or other intellectual function in the absence of metabolic, psychiatric, pharmacological or toxic causes.
1 3
Seizures (not hypertensive)
Clinical or EEG evidence of aberrant electrical activity in the brain
3 9
Stroke
Focal neurological signs lasting >24 hours due to a CNS vascular event
3 9
Spinal cord lesion
Clinical or imaging evidence of spinal cord involvement
3 9
Cranial nerve palsy
Clinical evidence of cranial nerve palsy – score VIII nerve palsy as sensorineural hearing loss, do not score ocular palsies if they secondary to pressure effects
3 6
Sensory peripheral neuropathy
Objective sensory deficit in a non-dermatomal distribution
3 6
Mononeuritis multiplex
Single or multiple specific motor nerve palsies
3 9

Persistent scores can range from 0 to 33 whilst the New/Worse scores can range from 0 to 63.

There are several rules for scoring BVAS:

Disease manifestations are to be scored only when they are attributable to active vasculitis. If there is reasonable evidence a manifestation is likely attributable to another aetiology (e.g. infection, other co-morbidity), it should not be scored.

Persistent disease scoring should be used for all manifestations due to active (but not new or worsened) vasculitis.

Specialist opinion, or the results of laboratory or imaging investigations will be required for some items.

The items of serum creatinine in section 8. “Renal” should be scored only on the first visit.

There are several items, i.e. Nodules or cavities that are not compatible with persistent disease and can only be scored under new/worse scoring.

When the BVAS is assessed for the first time in that patient or any of the features have started or deteriorated in the past month, please use the new/worse scoring.

Features persisting for > 3 months represent damage rather than activity and should be scored on a Vasculitis Damage Index (VDI) form and not on a BVAS form.

 

About the original study

With the 2008 study, Mukhtyar et al. set out to modify and validate version 3 of the BVAS in patients with systemic vasculitis in a prospective cross-sectional study of 313 patients. The number of items was modified from 66 to 56.

The BVAS v3 correlated with treatment decision (Spearman's r(s) = 0.66, 95% CI 0.59 to 0.72). The intraclass correlation coefficients for reproducibility and repeatability were 0.96 (95% CI 0.95 to 0.97) and 0.96 (95% CI 0.92 to 0.97), respectively.

The BVAS v3 demonstrates convergence with BVAS v2, treatment decision, physician global assessment of disease activity, vasculitis activity index and C-reactive protein results and is a score that is easily reproducible and sensitive to change.

 

References

Version 3

Mukhtyar C, et al. Modification and validation of the Birmingham Vasculitis Activity Score (version 3) Ann Rheum Dis. 2008;Dec 3.

Version 2

Luqmani, RA, et al. Disease assessment and management of the vasculitides. Baillieres Clin Rheumatol 1997;11(2): 423-46.

Version 1

Luqmani, RA, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM 1994;87(11):671-8.


Specialty: Rheumatology

Objective: Assessment

No. Of Items: 56

Year Of Study: 2008

Abbreviation: BVAS

Article By: Denise Nedea

Published On: April 23, 2020 · 12:00 AM

Last Checked: April 23, 2020

Next Review: April 23, 2025