Glossary and scoring for BVAS 2003. GENERAL ... - Rare Disease

Pleural effusion/pleurisyPleural pain and/or friction rub on clinical assessment or new onset of radiologicallyconfirmed pleural effusion. Other causes (e.g. infection, malignancy) should beexcludedBVAS BVAS new/persistent worse2 4Infiltrate Detected by CXR or CT scan. Other causes (infection) should be excluded 2 4EndobronchialinvolvementMassivehaemoptysis/alveolarhaemorrhageEndobronchial pseudotumor or ulcerative lesions. Other causes such as infection ormalignancy should be excluded. NB: smooth stenotic lesions to be included in VDI;subglottic lesions to be recorded in the ENT section.Major pulmonary bleeding, with shifting pulmonary infiltrates; other causes ofbleeding should be excluded if possible2 44 6Respiratory failure Dyspnoea which is sufficiently severe as to require artificial ventilation 4 66. Cardiovascular Maximum scores 3 6Loss of pulsesLoss of pulses in any vessel detected clinically; this may include loss of pulses1 4leading to threatened loss of limbValvular heart diseaseSignificant valve abnormalities in the aortic mitral or pulmonary valves detectedclinically or echocardiographically.2 4Pericarditis Pericardial pain &/or friction rub on clinical assessment. 1 3Ischaemic cardiac pain Typical clinical history of cardiac pain leading to myocardial infarction or angina. 2 4Consider the possibility of more common causes (eg atherosclerosis)CardiomyopathySignificant impairment of cardiac function due to poor ventricular wall motionconfirmed on echocardiography3 6Congestive cardiac failure Heart failure by history or clinical examination 3 67. Abdominal Maximum scores 4 9PeritonismAcute abdominal pain with peritonism/peritonitis due to perforation/infarction of smallbowel, appendix or gallbladder etc., or acute pancreatitis confirmed byradiology/surgery/elevated amylase3 9Bloody diarrhoea Of recent onset; inflammatory bowel disease and infectious causes excluded. 3 9Ischaemic abdominal pain Severe abdominal pain with typical features of ischaemia confirmed by imaging or atsurgery, with typical appearances of aneursyms or abnormal vasculaturecharacteristic of vasculitis.2 68. Renal Maximum scores 6 12Hypertension Diastolic BP>95, accelerated or not, with or without retinal changes. 1 4Proteinuria >1+ on urinalysis; >0.2g/24 hours Infection should be excluded. 2 410 or more RBC per hpf ( high power field ), excluding urinary infection and urinaryHaematurialithiasis (stone) 3 6Creatinine 125-249 Serum creatinine values 125-249 µmol/l at first assessment only. 2 4Creatinine 250-499 Serum creatinine values 250-499 µmol/l at first assessment only. 3 6Creatinine ≥ 500 Serum creatinine values 500 µmol/l or greater at first assessment only. 4 8Rise in creatinine > 30% Significant deterioration in renal function attributable to active vasculitis. 6or creatinine clearance fall> 25%9. Nervous system Maximum scores 6 9Headache New, unaccustomed & persistent headache 1 1MeningitisOrganic confusionSeizures (nothypertensive)Severe headache with neck stiffness ascribed to inflammatory meningitis afterexcluding infection/bleedingImpaired orientation, memory or other intellectual function in the absence ofmetabolic, psychiatric, pharmacological or toxic causes.Paroxysmal electrical discharges in the brain & producing characteristic physicalchanges including tonic & clonic movements & certain behavioural changes.1 31 33 9StrokeCord lesionCranial nerve palsySensory peripheralneuropathyCerebrovascular accident resulting in focal neurological signs such as paresis,weakness, etc. A stroke due to other causes (eg atherosclerosis) should beconsidered & appropriate neurological advice is recommendedTransverse myelitis with lower extremity weakness or sensory loss (usually with adetectable sensory level) with loss of sphincter control (rectal & urinary bladder).Facial nerve palsy, recurrent nerve palsy, oculomotor nerve palsy etc. excludingsensorineural hearing loss and ophthalmic symptoms due to inflammationSensory neuropathy resulting in glove &/or stocking distribution of sensory loss.Other causes should be excluded (e.g. idiopathic, metabolic, vitamin deficiencies,infectious, toxic, hereditary).3 93 93 63 6Motor mononeuritismultiplexSimultaneous neuritis of many peripheral nerves, only scored if motor involvement.Other causes should be excluded (diabetes, sarcoidosis, carcinoma, amyloidosis).10. OTHER Other feature of active vasculitis-please describe3 9