Birmingham Vasculitis Activity Score (version 3)

Select items only if attributable to active vasculitis. If there are no abnormalities in a section, please select ‘None’ for that organ-system.

Is this the patient’s first assessment? Yes No

1. General

None Features described below

  • Myalgia Pain in the muscles
  • Arthralgia / arthritis Pain in the joints or joint inflammation
  • Fever ≥38°C Documented oral / axillary temperature. If rectal temperature is measured, raise threshold to 38.5°C
  • Weight loss ≥2 kg Loss of dry body weight without dieting

2. Cutaneous

None Features described below

  • Infarct Area of tissue necrosis or splinter haemorrhages
  • Purpura Subcutaneous or submucosal haemorrhage in the absence of trauma
  • Ulcer A disruption in the continuity of the skin
  • Gangrene Extensive tissue necrosis
  • Other skin vasculitis Livedo reticularis, subcutaneous nodules, erythema nodosum, etc

3. Mucous membranes / eyes

None Features described below

  • Mouth ulcers Aphthous stomatitis, deep ulcers, strawberry gingival hyperplasia
  • Genital ulcers Ulcers on the genitalia or perineum
  • Adnexal inflammation Salivary or lacrimal gland inflammation
  • Significant proptosis >2 mm protrusion of the eyeball
  • Scleritis / Episcleritis Inflammation of the sclera
  • Conjunctivitis / Blepharitis / Keratitis Inflammation of the conjunctiva, eyelids or cornea - but not due to sicca syndrome
  • Blurred vision Deterioration of visual acuity from previous or baseline
  • Sudden visual loss Acute loss of vision
  • Uveitis Inflammation of the uvea (iris, ciliary body, choroid)
  • 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

4. ENT

None Features described below

  • 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
  • Paranasal sinus involvement Tenderness or pain over paranasal sinuses - usually confirmed by imaging
  • Subglottic stenosis Stridor or hoarseness due to inflammation and narrowing of the subglottic area observed by laryngoscopy
  • Conductive hearing loss Hearing loss due to middle ear involvement, usually confirmed by audiometry
  • Sensorineural hearing loss Hearing loss due to auditory nerve or cochlear damage - usually confirmed by audiometry

5. Chest

None Features described below

  • Wheeze Wheeze on clinical examination
  • Nodules or cavities New lesions detected on imaging
  • Pleural effusion / pleurisy Pleural pain and/or friction rub on clinical assessment; radiologically confirmed pleural effusion
  • Infiltrate Detected on chest X-ray or CT scan
  • 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.
  • Massive haemoptysis / alveolar haemorrhage Major pulmonary bleeding, with shifting pulmonary infiltrates
  • Respiratory failure The need for artificial ventilation

6. Cardiovascular

None Features described below

  • Loss of pulses Clinical absence of peripheral arterial pulsation in any limb
  • Valvular heart disease Clinical or echo detection of aortic / mitral / pulmonary valve involvement
  • Pericarditis Pericardial pain / friction rub on clinical assessment
  • Ischaemic cardiac pain Typical clinical history of cardiac pain leading to myocardial infarction or angina
  • Cardiomyopathy Significant impairment of cardiac function due to poor ventricular wall motion confirmed on echocardiography
  • Congestive cardiac failure Heart failure by history or clinical examination

7. Abdominal

None Features described below

  • Peritonitis Typical abdominal pain suggestive of peritoneal involvement
  • Bloody diarrhoea Of recent onset
  • Ischaemic abdominal pain Typical abdominal pain suggestive of bowel ischaemia, confirmed by imaging or surgery

8. Renal

None Features described below

  • Hypertension Diastolic >95 mm Hg
  • Proteinuria >1+ on urinalysis or >0.2g/24 hours
  • Haematuria ≥10 RBCs/hpf ‘Moderate’ on urinalysis or ≥10 RBC per high power field, usually accompanied by red cell casts
  • Creatinine 125-249μ/L(1.41-2.82mg/dl) [Can only be scored on the first assessment] -
  • Creatinine 250-499 μ/L(2.83-5.64mg/dl) [Can only be scored on the first assessment] -
  • Creatinine ≥500 μ/L (≥5.66mg/dl) [Can only be scored on the first assessment] -
  • Rise in serum creatinine >30% or fall in creatinine clearance >25% Progressive worsening of renal function. Can be used at each assessment if the renal function has deteriorated from prior value

9. Nervous system

None Features described below

  • Headache Unaccustomed & persistent headache
  • Meningitis Clinical evidence of meningism
  • Organic confusion Impaired orientation, memory or other intellectual function in the absence of metabolic, psychiatric, pharmacological or toxic causes
  • Seizures (not hypertensive) Clinical or EEG evidence of aberrant electrical activity in the brain
  • Stroke Focal neurological signs lasting >24 hours due to a CNS vascular event
  • Spinal cord lesion Clinical or imaging evidence of spinal cord involvement
  • 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
  • Sensory peripheral neuropathy Objective sensory deficit in a non-dermatomal distribution
  • Mononeuritis multiplex Single or multiple specific motor nerve palsies

Persistence

New / Worse Persistent

Please only select Persistent if all disease features have been present for > 1 month. If any of the features have started or deteriorated in the past month, please select New / Worse. New / Worse will also be selected automatically if this is the patient’s first visit or if you have picked a feature that is new by definition (sudden visual loss, nodules or cavities, significant rise in serum creatinine or fall in creatinine clearance). Features perisisting 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.

Summary

You have not noted any disease features.

Help

If this is patient’s first assessment or any of the features have started or deteriorated in the past month, please select New / Worse. Please only select Persistent if all disease features have been present for > 1 month. Features perisisting 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 (unless this is patient’s first assessment; then all items should be scored on BVAS form regardless how long they have been present).

Each section must be completed by noting one or more disease features or marking none if that organ-system is unaffected. Each feature contributes to the overall score with a maximum value for each system, indicated by a + after score shown in the section title.

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Background Information

The Birmingham Vasculitis Activity Score (BVAS) is a method for assessing the activity of vasculitis. Note that scoring ranges are higher when any of the features are new or worse. Creatinine levels can be scored at patient’s first assessment only.

References:

  1. Luqmani et al (1994). "Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis." QJM 87(11):671-8
  2. Luqmani et al. (1997). "Disease assessment and management of the vasculitides." Baillieres Clin Rheumatol 11(2): 423-46;
  3. Mukhtyar et al (2009). "Modification and validation of the Birmingham Vasculitis Activity Score (version 3) ARD 2009 68:1827