HSP in Children Self limited

20% long standing renal

involvement. Of that 20%, 7%
need HD Children with renal
in...
HSP in Adults NO
comparable incidence in
adults Overall 26% mortality
in adults Knowledge of
natural history in a...
Pathogenesis Remains
unknown; however, it is
generally considered
Immune complex-mediated
disease characterized by ...
C1GALT1 polymorphisms are
associated with Henoch
Schnlein purpura nephritis

the G allele and GG genotype

of 1365A/G...
Clinical feature Rieu and
Noel reported renal
involvement in 33% of
children and 63% of adults
with HSP A character...
Renal InvolvementChildren Only 1-5% progress
to ESRD. 10-50% of children
get microscopic hematuria,
mild GN, and ...
Renal Involvement-Adults
11% on HD 10-30% ESRD at
15 years 27% CrCl <50%
50% persistent hematuria
47% moder...
Children versus Adults 95
adults and 57 children, 5 year

follow up Crescents and

nephrotic range proteinuria
equal i...
Renal Transplant after HSP
35% recurrence 11% graft
loss Meulders et al
Diagnostic criteria Palpable
purpura (mandatory) in the
presence of at least one of the
following four features:
Diff...
What constitutes renal
involvement? Isolated
Haematuria Isolated
Proteinuria Nephrotic
syndrome Nephritic synd...
What investigations if renal
involvement? Primary
Investigations U&E, HCO3,

creatinine Albumin urine

Protein:Creatinin...
HSP Onset of nephritis
Time of onset of urinary
abnormalities after the
diagnosis of HSP Weeks after
HSP diagnosis 1...
Indications for renal biopsy
Acute renal
impairment/nephritic
syndrome at presentation
Nephrotic syndrome with
norma...
Henoch- Schnlein
Nephritis histological
classification ISKDC Class
Mesangial change Crescents
Frequency % (n = 355) ...

Histology and the normal

glomerulus
Normal glomerulus
Moderate mesangial
expansion class 2 mild.
Mesangial proliferation and
crescent formation Foc...
Immunofluorescence
microscopy. There is granular
staining for IgA in mesangial
regions and segmentally
along capillary ...
Electron microscopy.
There are electron-dense
deposits in the mesangium,
and segmentally in
subendothelial locations.
What treatments can be
used for HSP Nephritis?

Immunosuppressants
Steroids IV MP po
Prednisolone Cyclophos...
No adequate evidence
No RCTs for treatment of HSP
nephritis The treatment of
HSP nephritis is controversial,
and ...
Current RHSC(G) guideline
HSP Class Management I & II
No immunosuppressant
treatment routinely ACEi if
persistent prote...
Will steroids prevent renal
disease in HSP? No!
ChartapisakW, Opastirakul S,
Willis NS, Craig JC, Hodson EM.
ADC, 2008...

Will steroids improve

outcome for acute mild renal
symptoms? RCT early
steroid in HSP Sub group
analysis 71 with...
Recommended follow-up
BP & urine dipstix for week
1-6 weekly Week 7-24
monthly Narchi H Arch Dis
Child 90:916-20...
The adult kidney 24 years
after childhood HSP: a
retrospective cohort study.
Clinical presentation at onset
of Henoch - ...
The adult kidney 24 years
after childhood HSP: a
retrospective cohort study.

Onset Grade Number of

patients Good outcome...
Discuss with Nephrologist
Hypertension Abnormal
renal function Macroscopic
haematuria > 5 days
Persisting prot...
Summary HSP is the
commonest vasculitis of
childhood Renal involvement
is frequent Significant renal
disease is l...
Thank you
Detection and referral of
patients with Henoch
Schnlein purpura nephritis
(adapted from local guidelines
developed by Dr...
HSP nephritis

HSP nephritis