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A patient with POEMS syndrome: the pathology of glomerular microangiopathy.
[systemic capillary leak syndrome]
POEMS
syndrome
(
polyneuropathy
,
organomegaly
,
endocrinopathy
,
monoclonal
gammopathy
and
skin
changes
)
is
potentially
fatal
multisystem
disorder
but
its
renal
complication
has
often
been
overlooked
because
renal
involvement
is
not
necessarily
included
in
the
diagnostic
criteria
of
POEMS
.
This
report
documents
the
patient
with
POEMS
syndrome
with
long
-term
renal
histopathological
changes
evaluated
by
renal
biopsy
.
A
32
-
year
-old
Japanese
woman
presented
with
symptoms
consistent
with
POEMS
syndrome
associated
with
proteinuria
and
IgA-λ
type
monoclonal
gammopathy
.
Initial
renal
biopsy
for
confirmation
of
diagnosis
revealed
the
proliferation
of
glomerular
capillary
loops
located
in
the
expanded
mesangial
matrices
associated
with
glomerular
enlargement
.
Electron
microscopy
examination
of
the
renal
biopsy
revealed
the
presence
of
double
contoured
glomerular
basement
membrane
containing
peculiar
fibrillary
structure
.
The
patient
was
therefore
initially
diagnosed
as
membranoproliferative
glomerulonephritis
(
MPGN
)
-
like
lesion
without
any
significant
immunoglobulins
and
complements
deposition
.
The
patient
was
subsequently
admitted
to
hospital
on
five
occasions
due
to
renal
dysfunction
and
anasarca
for
the
next
four
years
of
her
clinical
course
.
The
severity
of
anasarca
was
correlated
mainly
with
serum
titer
of
vascular
endothelial
growth
factor
(
VEGF
)
during
this
period
.
Acute
renal
failure
occurred
at
the
last
admission
and
the
second
biopsy
was
performed
.
An
increased
mesangial
matrix
and
frequent
global
sclerosis
of
the
glomeruli
with
arteriolosclerosis
was
noted
in
this
second
biopsy
compared
to
the
first
one
.
These
findings
of
renal
biopsies
suggest
that
the
glomerular
microangiopathy
of
POEMS
syndrome
may
occur
in
the
context
of
systemic
capillary
leak
syndrome
superimposed
on
chronic
endothelial
injury
.
Diseases
Validation
Diseases presenting
"renal biopsies"
symptom
familial mediterranean fever
sneddon syndrome
systemic capillary leak syndrome
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