vasculitis assessment for MYPAN Pavla Dolezalova Paediatric - - PowerPoint PPT Presentation

Overview of the training in vasculitis assessment for MYPAN

Pavla Dolezalova Paediatric Rheumatology Unit General University Hospital and Charles University in Prague Czech Republic

 Theoretical background

 E-learning:

 Disease activity (PVAS)  Disease damage (PVDI)

 Training manual:

 PVAS instructions, 3 example cases with answers  PVDI instructions, 3 example cases with answers

 Homework I - basic

 5 PVAS and 5 PVDI training cases  Homework II – advanced  5 PVAS and 5 PVDI training cases

 Scoresheets

 Use bold black pen to cross the boxes  Scanned scoresheets to be sent to dolezalova.pavla@vfn.cz

 Certification requirement

 85% mean agreement with correct answers for each set of

training cases

Paediatric vasculitis activity assessment: PVAS - Paediatric Vasculitis Activity Score

MYPAN Investigator Training

Disease activity assessment

 Vasculitis activity concept

 Reversible manifestations resulting directly from

current vasculitis

 Exclusion of

 co-morbidities such as infection  disease damage

 Drives therapeutic decision-making (reflects

intention-to-treat)

PVAS

 Scoresheet to document

clinically active vasculitis requiring treatment

 Set of 64 items in 9 organ-

based systems

 Items weighted according

to their severity

 The total score (0-63)

indicates disease activity at the time of scoring

PVAS

Webb, E. M. et al. Am. J. Roentgenol. 2000;174:1315-1321

Marom, E. M. et al. Am. J. Roentgenol. 2001;176:713-717

Questions to be asked

 1. Is the abnormality present?  2. Can it be attributed to active vasculitis?  3. If so, is it newly present or worse or does it

reflect low grade grumbling disease activity?

PVAS

Scored items:

 “Active”

 Tick box only if abnormality due to active vasculitis is newly present or worse

• ver the last 4 weeks or persists for less than 3 months.

 „Persistent disease only“

 Tick box only if ALL active items are persistent and represent

smouldering/low grade/grumbling disease, and there are no new/worse features

PVAS glossary and scoring

 Maximum PVAS persistent score: 33  Maximum PVAS new/worse score: 63

Scoring a patient

 Features of active vasculitis

 Tick the box „Active“

 If any item represents new or worse disease

 “Persistent disease only” box is not ticked

 If all ticked items have been present

for ≤3 months, but NONE new or worse within the last 28 days

 “Persistent disease only” box is ticked

 No active disease

 Tick the box „None“ for the system if

 No active disease items present  An item present for ˃ 3 months and you

you would not increase immunosupression

 A patient reports having had a symptom more than 4 weeks ago but it is

not present at the time of scoring



Scoring a new patient

 Usually at the time of diagnosis  Prior to the immunosuppressive treatment is

started

 All active vasculitis items score as New/Worse  Items may have been present for over 3 months

Scoring a patient with some new and some persistent items

 All active disease items

score as New/Worse

 even if there is only one item

that is new/worse

 Intention to treat the

patient

 on the basis of at least one of

the ticked items

         

Next steps

 Please read carefully the PVAS section of the

Instructions manual

 Remember

 Do not forget to tick the box „None“ for organ systems that

are NOT affected

 The box „Persistent disease only“ should be ticked only if

NONE of the active vasculitis items has been new or worse

• ver the past 4 weeks

 Test yourself

 Score 3 example PVAS cases  Review correct answers

 Start with scoring the Homework I training cases only

when you feel confident with the PVAS principles

 Contact Pavla Dolezalova with any questions on PVAS