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 over 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 over 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
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