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 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