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

• f 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

• f 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

• f 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.