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.
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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