Paediatric vasculitis damage assessment: PVDI - Paediatric - PowerPoint PPT Presentation

Paediatric vasculitis damage assessment: PVDI - Paediatric Vasculitis Damage Index Pavla Dolezalova Paediatric Rheumatology Unit General University Hospital and Charles University in Prague Czech Republic

PVDI development  Based on VDI  Delphi and NGT used to reach the current PVDI version  Items re-grouped to 9 organ systems (peripheral vascular system deleted)  Retains original VDI items (64) + 8 paediatric ones  Distinguishes items currently present and those that have resolved  Glossary adapted for the paediatric use  School absence assessment added  Formal validation not yet completed

PVDI  9 organ based systems + Other section  Each item scores 1 point  Item definitions in the PVDI Glossary

Paediatric Vasculitis Damage Index Glossary  Disease damage is defined by duration of the sign/symptom as at least 3 months any time since the onset of vasculitis  The item does not need to be present at the time of scoring (NLP – No Longer Present)  Some of the items and their description are very similar to those of the PVAS  DURATION and NEED FOR INCREASED IMMUNOSUPPRESSIVE TREATMENT are the main distinctions

 Damage is defined as irreversible in principle  This applies also to features that may have disappeared by the time of your current evaluation  “irreversibility“  a long-standing (3 months arbitrarily chosen) general impact of an item on patient ´ s life  not necessarily causing a life-long persistent physical sign (“scarr”)  E.g. failure to thrive or delayed puberty = NLP  Majority of items represent true scarring  E.g. tissue loss, nasal bridge collapse, any surgery = always present  PVDI list can only remain stable or deteriorate over time.  The PVDI does not improve with time

The PVDI items may be present:  1. As a direct consequence of vasculitis disease activity  2. As a consequence of vasculitis therapy  3. Or as a consequence of related or unrelated comorbidity that develops or deteriorates after the onset of vasculitis

Features of damage to score as „ Present “  present for ≥3 months and are still present at the time  of the assessment Features of damage to score as NLP  ( “No Longer Present” ) present in the past starting after the vasculitis onset  lasting for ≥3 months  resolving by the time of the assessment  Note also that you cannot score any single  item as “present” and “NLP” at the same time.

 During long-term follow-up some damage items may move from the “Present” box to the “NLP” (No Longer Present) box  The PVDI score can only remain stable or deteriorate over time  both “NLP” and “Present” damage items carry a numeric score of 1 and no item can be scored under both at once

OTHER section  Lists general items that are not organ-based  Additional items can be added in handwriting  based on their clinical significance as assessed by the physician and/or the patient  E.g. renal transplantation or insertion of a dialysis catheter

Repeat episodes  In the case of blindness, myocardial infarction, loss of pulses, major tissue loss, or stroke, repeat episodes may be recorded (in Other section)  ONLY if at least 3 months apart  For example, blindness in the left eye would score if present for ≥3 months; if blindness in the right eye then developed, this would only score on the PVDI when present for ≥3 months.

„Double“ scoring  The same pathological feature  cannot be scored in more than one organ/system section  Example: optic atrophy - cranial nerve lesion, atrophy of interosseal hand muscles associated with ulnar neuropathy  Each feature of damage is scored, but not more than once except in cases in which the same item of damage has occurred in the contralateral organ.

„Multiple“ scoring  Each pathological event causing damage  May be scored by one or more damage items within that system category  This may reflect its clinical importance/severity  E.g. cataract with/out visual impairment, pulmonary fibrosis with/out pulmonary hypertension, coronary stenosis with/out myocardial infarction etc.

PVAS vs. PVDI Some items may appear similar in PVAS and PVDI (eg. Bowel ischaemia or Stroke)  Count as Activity item if  Present at the initial assessment (regardless their duration)  Present for less than 3 months  If the Bowel ischemia or Stroke has occurred within 3 months of the last PVAS and PVDI assessment, it is scored in PVAS as active disease, not as damage in the PVDI  Count as Damage item if  Present for longer than 3 months any time since disease onset  Items should NOT be scored on both the PVAS and PVDI: events are either disease activity or damage, but never both at the same time.