Paediatric Vasculitis Damage Assessment Training: PVDI Basic Training Cases 1-5
Homework I
PVDI 01
A 13-year-old boy returns for follow up. He originally developed GPA with ENT involvement,
epistaxis, nasal crusting and discharge with haemoptysis, pulmonary nodules and a necrotizing
cutaneous vasculitis four years prior to this review visit. These features all improved with treatment
within two months. He has never had renal involvement. He has been chronically breathless for at
least twelve months, with documented reduction of gas transfer factor to 45% predicted on pulmonary
function and reduced total lung capacity. Imaging studies show pulmonary fibrosis. Since his last
assessment 3 months ago he has managed to attend school with help from the learning support unit 4
days a week.
Please score his PVDI and school absence.
PVDI 02
A 16-year-old girl has a two year history of microscopic polyangiitis. She originally had cutaneous
vasculitis, pulmonary haemorrhage with haemoptysis and rapidly progressing glomerulonephritis, with
an active urinary sediment. A renal biopsy confirmed a pauci-immune nephritis. Her cutaneous and
pulmonary manifestations had resolved with intensive immunosupression with steroids and
cyclophosphamide, but her renal function deteriorated and she went into renal failure. She had a renal
transplant twelve months prior to the current visit. Six months after the diagnosis she developed
hypertension which was attributed to steroid use and required two antihypertensive drugs to control it
which she had been taking for 8 months. Her renal transplant was complicated by an episode of sepsis
and she subsequently developed bacterial endocarditis with large vegetations on her mitral valve.
These caused mitral incompetence and she subsequently (12 months ago) had an embolic dense right
hemiplegia from which she has made a good, although incomplete, recovery with mild right-sided
weakness that has remained stable since then. For the last six months she has been complaining of pain
in the right hip and MRI has confirmed the presence of avascular necrosis. Her last PVDI assessment
was 6 months ago and she missed just one day of school for the MRI.
Please score her PVDI and school absence.
PVDI 03
A 17-year-old young man with GPA attends for review. He presented at the age of 12 with a retro
orbital mass, a red eye and panophthalmitis associated with otitis media and nasal crusting and
discharge. A biopsy from the retro orbital tissue confirmed the presence of active granulomatous
inflammation. He has never had any renal or lung involvement. Aggressive cytotoxic therapy and
steroids failed to improve his vision or pain in the right eye and a CT scan showed some orbital wall
destruction within the first six months of diagnosis. He went blind in the eye and it was eventually
enucleated two years ago. In the first year of diagnosis he had steroid induced pancreatitis associated
with chronic malabsorption that persisted for more than a year. Cyclophosphamide unfortunately
induced severe marrow failure, which persisted for three weeks. His last review was 6 months ago over
which time he has had two upper respiratory tract infections resulting in missing 15 days of school in
total.
Please score his PVDI and school absence.
PVDI 04
A 17-year-old young woman presents for review. She had a diagnosis of GPA established at the age
of 8 years having presented with hearing loss due to otitis media, nasal discharge, epistaxis and nasal
crusting with an associated haematuria and proteinuria. Renal biopsy confirmed the presence of
glomerulonephritis and she was treated with high dose cyclophosphamide and prednisolone. A year
after initiating steroids she fell and sustained a low trauma fracture of her left femur due to steroidinduced osteoporosis confirmed by bone densitometry. Four years ago she had breathlessness on
minimal exertion and ankle oedema. She was diagnosed as having an ischaemic cardiomyopathy and
breathlessness which persisted for 4 months despite good control of heart failure with diuretics. Two
Paediatric Vasculitis Damage Assessment Training: PVDI Basic Training Cases 1-5
Homework I
years previously she had a disease relapse with abdominal pain diagnosed as due to gut vasculitis with
infarction; this required resection of 30 cm of small bowel. She also had a deterioration of renal
function with GFR fall to less than 40% of the lower limit of normal and has remained at that level
since then. She is not on dialysis yet but it is being contemplated should her renal function deteriorate
further. Since that flare she has been hypertensive requiring medication for it. She was last reviewed
for PVDI 3 months ago and has missed 2 days of school.
Please score her PVDI and school absence.
PVDI 05
A 17-year-old female presents for review. Her GPA started seven years ago with sudden onset of
deafness, fever and sore throat. The hearing loss was found to be bilateral and sensorineural. There
has been no subsequent improvement in her hearing. She developed subglottic stenosis, which was
managed with methotrexate and prednisolone and resolved in two months without the need for a
tracheostomy. Three years after presentation, she had a relapse with worsening nasal symptoms with
crusting, discharge and epistaxis. A CT showed nasal sinus destruction. She also developed
hypertension at the same time and has since required regular anti hypertensive medications (at least
two). At the time of her relapse she developed a motor mononeuritis multiplex, purpuric skin vasculitis
and gastrointestinal haemorrhage which was found on endoscopy to be due to multiple infarcts. She
required gut resection of 25cm of small bowel and was treated with cyclophosphamide and
prednisolone. Her mononeuritis improved but she is left with residual right foot drop and wasting of
the left hand interosseus muscles from an ulnar nerve lesion. Over the last 3 months since the last PVDI
she has missed just 2 days of school.
Please score her PVDI and school absence.