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Cystic kidney disease in a patient with systemic toxicity from long-term D-penicillamine use.
[cystinuria]
D-
penicillamine
,
used
to
treat
cystinuria
,
is
known
to
cause
impaired
collagen
deposition
and
dysfunction
in
elastic
fibers
.
D-
penicillamine
also
has
been
associated
with
glomerular
abnormalities
,
typically
membranous
glomerulonephritis
.
We
describe
a
patient
with
severe
bilateral
cystic
kidney
disease
that
developed
after
long
-term
D-
penicillamine
use
for
treatment
of
cystinuria
.
The
cysts
in
the
kidneys
were
noted
during
an
evaluation
for
acute
kidney
injury
.
The
patient
had
no
evidence
of
cysts
on
prior
renal
imaging
at
a
time
when
his
kidney
function
was
normal
.
Simultaneously
,
he
presented
with
multiorgan
manifestations
of
D-
penicillamine
toxicity
,
including
the
skin
findings
of
cutix
laxa
and
elastosis
perforans
serpiginosa
.
Consequently
,
D-
penicillamine
treatment
was
discontinued
,
after
which
the
progression
of
cystic
kidney
disease
gradually
ceased
,
along
with
the
other
systemic
manifestations
of
toxicity
.
To
our
knowledge
,
this
is
the
first
report
of
cystic
kidney
disease
associated
with
and
perhaps
caused
by
long
-term
d-penicillamine
therapy
.
The
proposed
mechanism
of
cyst
formation
is
the
malfunction
of
the
extracellular
matrix
of
the
kidney
by
d-penicillamine
that
leads
to
an
impaired
repair
process
after
kidney
injury
.
