Manual of operations for assessment vasculitis using BVAS Dr Anthony Lee, Ms Cathy Hall and Dr Rashid Lugmani Rheumatology Department, University of Edinburgh UK, Section 1- Introduction Welcome to this brief review of assessment of vasculitis using the BVAS (Birmingham Vasculitis Activity Score). This assessment method is a clinical evaluation tool which has been standardised for the management of patients with systemic vasculitis. It is important to standardise how we use this tool so that we agree definitions of disease activity. This brief summary is designed to help you understand the concepts of BVAS as well as providing you with some simple practical exercises on completing BVAS. before applying it to patients with systemic vasculitis. A full version of this manual is available on line at the following address: hup: ‘homepages ed.ae.uk/rluq/vasculitistraining htm 1.1 Aims and Objectives Systemic vasculitis is now viewed as chronic disease rather than a fatal condition due to improvement in survival by treating with chemotherapy. Death as the main study come is no longer appropriate, standardized methods of assessment of morbidity are available to accurately measuring outcome of vasculitis and its effects, which consists of: * current disease activity « increasing damage, which consists of non-healing scars developing as a consequence to initial disease, eg renal failure, hypertension ‘There are three aspects of assessment tools used for outcome of clinical trials in vasculitis 1. BVAS-used to record current disease activity 2. VDI (vasculitis damage index)-used to record damage as a result of vasculitis and its treatment 3. SF-36 patient questionnaire-used to measure physical and mental function 1.2 Methods This training programme is designed to introduce you to BVAS so that we can standardise the way in which we collect data in clinical trials. 1.3 Assessment of Vasculitis Dist se Activity BVAS (Birmingham Vasculitis Activity Score) It is designed to document new or worsening clinically active vasculitis that would be likely to require treatment, after exclusion of other causes such as infection, hypertension, etc. It consists of a set of items divided into nine organ based systems, The scoring sheet records the presence or absence of each item. Each item is weighted and a maximum total score applied to each system. The total score on all nine systems gives an indication of the current disease activity. 1.4. Practical Notes on how to Complete BVAS. VAS for EUVAS studies 2sows Todt from Lagan RA” Bacon PA, Moon RT al TOI) BIAS emi nT aR war Wed BF OTT‘Three questions should be asked of each item on the scoring sheet on completion, Is the abnormality present? Can it be attributed to active vasculitis? Ifit can be attributed to active vasculitis, is it newly present or worse or does it simply reflect, low grade grumbling disease activity? ‘© Scoring all items that are present and attributable to vasculitis as New/Worse for patient presented for the first time. In this case the items can have been present for over 3 months or even longer © Scoring a patient for review with known disease if it is newly present or worse the New/Worse circle is ticked fit is present and has been present within the last 3 months (even if itis better now), the Persistent box is ticked iffno item is present, an item has been present for over 3 months, or a patient reports having a symptom but it is not present at the time of scoring on questioning or examination, the None box is ticked NB_ Ifyou tick a New/Worse circle there should be an intention to treat or to act on ticked items. eg. Ifa patient complains of new symptoms of joint pains and on examination there is no obvious joint swelling or tendemess and treatment is not fo be changed or commenced, the item should not be ticked. However if on examination there is obvious joint inflammation and it is intended to increase the dose of steroids the New/Worse circle should be ticked NB_ Asa general rule the persistent box is only ticked as stated above, if the item has been present and persistent within the last 3 months. There are however occasions when an item has been persistent for longer than 3 months but is obviously of concern and indicates “ongoing active disease that needs addressing, the Persistent box should be ticked eg. Nose bleeding or bloody nasal discharge which are still occurring on a regular basis. In this instance the Persistent box should be ticked as treatment will need to be monitored. If the Persistent box is not ticked, it would not be apparent that the disease is still active and the steroid dose might be wrongly reduced, which may lead to worsening of the symptoms 1.5. Overview of BVAS Disease features are scored only when they are due to active vasculitis, after exclusion of other obvious causes (eg infection, hypertension, etc). If the feature has occurred afresh or represents recent deterioration of status since last visit, itis scored in the New/Worse box. If abnormality indicates the presence of active vasculitis (but not new or worse), the Persistent box is ticked. For some occasions, further information (from specialist opinion or further tests) is required before entering some items. We would suggest that you leave these items blank, and once the information is available, please remember to fill in the information. ‘The data from the score sheet will be used to derive indices of disease activity as follows: BVAS. (new/worse) - this represent a score of new/worse disease activity attributable to vasculitis BVAS for EUVAS stucies 2wavs 2 “iodo rom Lagat BA. Bacon FA Moos RT Wa TA BAS a Wem WTO TS DAT FOTOBVAS.2 (persistent) - this represents a score of disease activity due to persisting or grumbling disease, which is neither new nor worse, compared to the previous assessment, Scores are calculated using the values given to each item; each section has a maximum score, corresponding to the total value for BVAS (new/worse) and BVAS (persistent) If the patient scores over the maximum number in a system, they only score the maximum score stipulated. Add the scores for each system together to get the final score ‘There are 2 separate final scores BVAS new/worse max score 63 BVAS persistent max score 33 The higher the scores the more active the disease BVAS for EUVAS studes 200 Mes fom Lagman RA, Bacon PA MaaeRT wat THAT BVIS a AERIS POOR ATS DoaT Med BT OTE‘Tick box only # abnormally is newly presont since ast assesement or worsein ihe | DEMOGRAPHY last fow woeks (use ihe Vascultis Damage Index, VDI to scove items of damage) Trial Number Tick box only i abnormality is dve to active (but not new or worse) vasculitis eto 7 Tick boxif more information (specialist opiniontests) is requested ray ® oralailary temperatures; rectal temperatures are 0.5°C higher investigator PERSISTENT NEWMORSE_| PERSISTENT [NEWTWORSE—| 7. GENERAL (none) z malaise a 2 rryalgia 0 e | arthraigiaiartnts i S ‘6. CHEST Ginone) ° headache a ° | persistent cough a g fever (<38.5°0)% o ° Syspnoea or wheeze Q ° Pc)@ a ° Haemopiysismaemonnage aed ° chest radology performed 2 wt oss 24a) 2 no active vasculitis ° cules 0” avis ° a eee ears) ° ‘pleural effusionipleurisy ° inna 5 a Infitrate ° purpura eS 8 massive haemoptyss or o ° other skin vasculitis a Prseeii tiene leer a ° respirator faire 2 9° a I Sng |__mutioe cigt gangrene CARDIOVASCULAR ° 3. MUCOUS NEN. —T(none) | 2orgcireompetence 8 matieae co |_| fachoeme earciae pain 5 ° Maud A congestive cardiac falure oO ° genital ulcers 0 e | cardiology opiniontests 8 significant proptosis a ° no active vasculitis ° redeye-conjunctivts 0 ° pencardie a - episcertis a ° ‘myocardial infarvangina red eye- episce Oe a biured vsion o ° eroiomyopathy sudden visual loss a ° 7. ABDOMINAL Tineney phthalic opinion ° ‘severe abdominal pein o ° no active vasculitis ° bloody diarrhoea a] » ° surgical opinionfests Cad 8 no acive vascults 8 retinal exudates ‘ut pecorationinfarct iS ‘etna haemorrhage 2 ‘Beute pancreatitis ° RENAL (none) - Saal ac! ° Typerension dastoas) "8 9 eee a protoinuna >1+/>0.29/28h) o 2 Bloody nasal ischarge Raomatuse> 1+=10rem) 0 ° Nasal crusting 9 ° Geatnine 125-249 uot e aca a 6 Ceatine 500 um ° fect ee 5 7 se in creatinine >30% or fll a Hoarseness/stridor a ° in creatinine clearance>25% ENT opinion : & NERVOUS SYSTEM — Crone) no active vascultis ‘exganie contusionidernentia 9 Granulomatous ° seizures(not hypertensive) HI a sinusitis stoke a ei Conductive hearing loss ° ‘21d lesion fe S Sensorineura hesrng ° sensory peripheral toss | neuropathy ° oot ° ‘cranial nerve palsy 3 igoneron en motor mononeurts muttplex __O ° inflammation 70. OTHER E = BBVAS for EUVAS sues zamvoe_4 Woiiied from Taqman 4 Bacon PA Moos RT eal TOR) TAS one ag asc Duar Wed WF TTGLOSSARY for BVAS (ara sm nese oat te cate eyes or ach — —— Santeria inmeren ect Creenen Eres] iisorein” Site Sees geht whan byaomeeramear meee eel Sport sent Speen Wt Gara te seta kc mimucemenae Sencens Ske oueter'e ses erase tane ance ae | aera og else Soeaeet 7] ‘Deseo was, Remeber oa most stones, you wi'be abe cles he Gar aaatay [med pan hcae a sone Tae SERSSr a Lats Saeco as abe he creme | smecwu ton easier | Siniaferuersepunterneowecaseyevocsiysne | Rimarrcmmar| “eras acest Bettina ang ee Soot wae Shs ae os Tia avaioe Vo naot nem er a S Soper pocorn enannvey | usta athgty cornet poor DERWATION of BVH rewwors)BVAS2 grater cores. 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TTT Fa pts oh eee! ener mes | 7 ea TET RET EEE TT ewan toe ne sere] —T a | Ee eee | UORTETEET]| aera ete VAS or EUVAS suds ave Totiied fom Taqman BA Bacon PA Moon RT wal (0) BVIS a amie Reagan Daa Wed OT OTELea 39452 VASCULITIS ACTIVITY SCORE < Tick ox on abnormal is wowy pose sic as assess ch : Srose nthe eal ew weeks se he Vasculls Damage index Volto, + Subject ID: Sexe lems of damage) een “Tek box ery abeoralty due to acv (tro new wos) vazeutls 3. Date form T T Tx oxi mre information specs opiniones) recuesedeomploteds @ oralasiay temperatures rectal temperatures ae 0.5 C higher isan a PERSISTENT _ NEWIWORSE PERSISTENT —NEWMORSE 1. GENERAL Ti tnone} 5. CHEST Di tnone malice qg 9° arson cough Q 9 mye q 8 Sfepnoen or whaeze A 8 rsigarartis 5 8 Rooroptstshaemomnage 8 fesuathe 3 Chest radotoay sorted u meio, q 3 iuckte vase g fever (0650) nodules or caves wes =k) a 38 Patil ofesenneey 3 rate naemopysis heal haemorhage 2. CUTANEOUS D inone) respiratory flue a tare go Q purvura q 8 6. CARDIOVASCULAR 1 (none) Sinan vast tier | 8 sore naompetonce Q 9 sonarene o 3 percaraat pans q 8 Fal 3 gangrene a 8 (chemi capi a 8 congestive crs ale Cerdeloay opmiontests u osc vsoae 9 pecaras 8 3. MUCOUS MEMBRANESIEVES Di tncne) Feel nace 8 caronyepety south oars g 8 rl e's Sonar’ ponte a 8 7.ABDOMINAL (none) ted oyeconuncvs wee cic ce Sire eon 8 Bioday darmoea a 8 suas ua xs a 8 Surgical pions y ame opion sauce vases mo acive vaceulie g it peroratonintret 3 vet, Seu pancreatte inl xaos 8 : ‘eal haerorhage ° @. RENAL D tnone) hypertension asa 5) q 9 4. ENT D (none) protemaris> Yet pan) q 8 hamatura>4oP>torboi) a 8 Nasal obstnton g 2 Creatine 138-40 8 Bi rec care 8 eatnne 250486 rah 8 Sirus inotement 8 3 fae in eratnine > 30% or fal Hearing os 3 in creatine dearance > 25% ° Hoaranessstder Boe ° oacve vascuis g @.NERVOUS SYSTEM Cl [none] Gtncvebve sing ss 8 crganicconfsionterent o 9 Sersovineual eating oss 9 selaus (at hyperensie) 8 8 Scriicant Subgiteatammaton 8 soe Q 8 Sensory poreneral nevropthy o 8 Sonat pone palsy 0 8 ‘olor morons maton a 8 10. OTHER o °