2. Patient
profile
• 21-‐year-‐old
man
• Underlying
disease：denied
systemic
disease
• Past
history
– Nil.
(Drug
alergy：Nil.)
• Family
history
– DM
:
Father
3. • Chief
complaint
– Progressive
erythematous
to
violaceous
macules
and
coalescent
patches
over
lower
limbs
for
one
week
• Start
from
bilateral
lower
limbs
with
several
erythematous
papules
and
plaques
with
extension
(thighs,
buttocks)
13. EULAR/PRINTO/PRES
2010
• Palpable
purpura,
not
thrombocytopenic/petechiae
(mandatory)
+
≥
one
of
the
following
• 1.
Diffuse
abdominal
pain
+/-‐
• 2.
Histopathology:
typical
LCV
with
predominant
IgA
deposits
or
proliferative
glomerulonephritis
with
predominant
IgA
deposits
• 3.
Arthritis
or
arthralgias
• 4.
Renal
involvement
Autoimmunity Reviews 13 (2014) 355–358
15. Treatment
course
• Derm
OPD
:
skin
lesions
improved
a
lot
– arthralgia
subsided
– abdominal
fullness
– hematuria
(-‐)
– bloody
stool
(+)
– diarrhea
(+/-‐)
– Pathology：Leukocytoclastic
vasculitis
• GI
OPD：Arrange
EDG
for
GI
survey,
pending
• Derm
OPD：
Remove
stitches
• Neph
OPD：Pending
01/23
01/29
01/30
16. Effects
of
Corticosteroid
on
Henoch-‐Schönlein
Purpura
“Prevention
of
serious
kidney
disease
in
adults
”
focus
on
17. Background
• Excellent
prognosis
for
long-‐term
kidney
function
in
children
with
microscopic
or
macroscopic
hematuria
alone.
Those
with
nephrotic
syndrome
and
reduced
kidney
function
frequently
show
a
progressive
course
to
ESKD.
• Corticosteroid
is
commonly
used
in
the
acute
phase
of
HSP,
particularly
for
abdominal
pain.
• Controversy
to
whether
corticosteroids
can
prevent
the
development
of
kidney
involvement,
reduce
its
severity
or
both
in
HSP
18. • Patients:
adults
with
HSP
with
none
or
minor
kidney
disease
(microscopic
haematuria,
mild
proteinuria)
at
presentation.
• Intervetion:
Corticosteroid
• Comparision:
Placebo
• Outcome:
to
determine
the
benefits
and
harms
of
different
interventions
used
to
prevent
persistent
kidney
disease
in
HSP.
21. u Include：
• RCTs
that
compared
corticosteroids
to
placebo
in
HSP
• Patients
of
any
age
with
HSP
with
or
without
kidney
disease
manifestations
(microscopic
hematuria,
macroscopic
hematuria,
proteinuria,
nephrotic
syndrome,
acute
nephritic
syndrome,
reduced
function,
acute
kidney
failure).
u Exclude：
•
Patients
with
other
forms
of
primary
or
secondary
GN
such
as
IgA
nephropathy,
mesangiocapillary
GN,
membranous
GN,
systemic
lupus
erythematosus,
rapidly
progressive
GN
not
associated
with
HSP,
other
systemic
vasculitis.
27. No
benefits
for
persistent
kidney
disease
• Data
overall
and
at
specified
time
points
after
presentation
in
children
with
persistent
kidney
disease.
– Eight-‐year
follow-‐up
of
those
with
urinary
abnormalities
or
hypertension.
• The
use
of
prednisone
in
patients
with
severe
kidney
disease
(nephrotic
syndrome,
nephritic
syndrome,
reduced
kidney
function).
• Those
with
kidney
disease
at
1,
3,
6
months
suggests
that
prednisone
did
not
result
in
more
rapid
resolution
of
kidney
disease
overall.
28. No
benefits
for
persistent
kidney
disease
• There
remains
uncertainty
as
to
the
efficacy
of
prednisone
in
preventing
severe
HSP-‐associated
kidney
disease
1. small
numbers
of
events
resulting
in
wide
confidence
intervals
2. inadequate
definition
of
severe
disease
29. Abdominal
pain,
joint
pain?
• (Ronkainen
2006a)
reported
that
the
severity
and
duration
of
abdominal
pain
and
the
duration
of
joint
pain
were
significantly
less
severe
in
children
treated
with
prednisone.
34. Conclusions
• No
evidence
of
benefit
from
RCTs
for
the
use
of
short-‐term
prednisone
or
anti-‐platelet
agents
to
prevent
persistent
kidney
disease
of
HSP.
• Though
heparin
appeared
effective,
this
potentially
dangerous
therapy
is
not
justified
to
prevent
serious
kidney
disease
when
fewer
than
2%
of
children
with
HSP
develop
severe
kidney
disease.
• No
evidence
of
benefit
for
cyclophosphamide
treatment
of
HSP
and
severe
kidney
disease.
• Unclear
about
cyclosporin
and
mycophenolate
mofetil
for
treatment
of
HSP
and
severe
kidney
disease.
35. Implications
for
research
• Prednisone
therapy
has
a
role
in
children
with
risk
factors
for
developing
kidney
disease
including
older
age
(Shin
2006),
severe
abdominal
pain
(Ronkainen
2006a;
Shin
2006),
persistent
(Ronkainen
2006a;
Shin
2006)
or
recurrent
purpura
(Shin
2006)
so
that
a
further
RCT
in
this
group
of
children
might
be
warranted.
• Recruitment
to
a
placebo
controlled
RCT
may
be
difficult
36. Take
Home
Messeges
• Recent
years,
retrospective
review
study
reveals
that
the
use
of
steroid
therapy
at
presentation
of
HSP
is
not
indicated
for
the
prevention
of
persistent
kidney
disease.
• Evidence
of
benefits
are
found
in
patients
of
HSP
with
abdominal
pain
and
joint
pain.
• More
well-‐designed
researches
should
be
performed.
38. References
• Cochrane
Database
of
Systematic
Reviews
2015,
Issue
8.
Art.
No.:
CD005128.
“Interventions
for
preventing
and
treating
kidney
disease
in
Henoch-‐Schönlein
Purpura
(HSP)
(Review)”
• Autoimmunity
Reviews
13
(2014)
355–358
“The
diagnosis
and
classification
of
Henoch–Schönlein
purpura:
An
updated
review”
• Pediatr
Nephrol
(2012)
27:933–939
“Outcome
of
Henoch–Schönlein
purpura
8
years
after
treatment
with
a
placebo
or
prednisone
at
disease
onset”
