VOL.
No.
124,
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JOINT
CHANGES IN HEMOCHROMATOSIS* JACK
By
TWERSKY,
BROOKLYN,
A
SPECIFIC
arthropathy
idiopathic well
associated
hemochromatosis
established.3”0”
joint tes,
and
The
major
brown
roentgen signs
skin
radiologist
the correct genographic large
and
the
pigmentation.’ be the first
can
diagnosis
on
changes
REPORT
i.
V.B.
Case
i.
This * From
the
been
For
in
the
the
is
OF
a 6o
can
has
pain
i. (A and B) is characterized
Department
This
was
space
narrowing,
of roent-
and
female
with
of Radiology,
Degenerative by State
asymmetric University
changes joint of New
in both
tal
at
I-P
are present space York,
‘39
the
and
knees
has
and Medical
and
#{231} years
she
past
and
shoulders.
examination of the hands of all of the metacarpojoints (Fig. i, ii and B). by
prominent prominent findings 1st
1953.
pain
the
asymmetric
eburnation
3rd
had
of the
in the 2nd and 3rd M-P osteophytes. Similar and were present in the (R)
(I-P),
interphalangeal
proximal
I-P
prominent Center,
joint
subchondral
joints,
and
at all of the metacanpo-phalangeal
narrowing Downstate
For
characterized
bone (most joints), and less prominent
since
she
hands.
Roentgenographic revealed abnormalities phalangeal (M-P)
diabe-
hands
had
years
10
the
hand old
hemochromatosis
past of both
CASES
year
proven the
stiffness
to suggest
basis
M.D. YORK
biopsy
joints.
CASE
Fic.
findings
of cirrhosis,
NEW
with
Furthermore,
symptoms
precede
has
IDIOPATHIC
Brooklyn,
joints.
osteophytes. New
2nd disin the
York.
Jack
140
Twersky
MAY,
articular
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cified
cartilage
CASE
II.
sented
of the
3, A
(Fig.
both
hands
there
has
is a 64 year
a
io
year
and been
knee
was
also
cal-
who
pre-
B).
and
F.B.
with
(L)
1975
old male,
history
shoulders.
of
For
an increase
arthritis
the
in both
in pain
of
past
year hands.
Roentgenographic examination of both hands revealed abnormalities of all the M-P joints characterized by asymmetric narrowing of the joint spaces, and prominent osteophytes (predominantly 2nd and 3rd M-P joints) (Fig. 4, A and B). Both shoulder joints had prominent osteophytes
along
humeral cystic
head changes
humeral
FIG.
2.
Case
the head osis and bone,
Left
i.
to
(L) hand at the (R) shoulder had
of cortical and
cystic
There
is flattening
of
of the humerus, with subchondral scleran irregular surface of the subchondral
similar
shoulder
shoulder.
an
1st
aseptic
carpo-metacarpal
was
joint
of humeral
change
(Fig. 2). Both knees involving primarily
joint.
unremarkable.
decreased
border
necrosis.
of
the
The
space,
head
The
(L)
irregularity
with
sclerosis
subchondral
revealed chondrocalcinosis the menisci, although
bone
of the A and On unusual
the
and
and
margins with
articular
cartilage
the
basis form
of of
the
B). both
(Fig.
6,
roentgenograms,
osteoarthritis, was
for
then
biopsy
admitted revealed:
metamorphosis;
present
“i.
2.
in hepatic
;-Hemochromatosis.”
an
possibly
was
tissue
the
B).
hemochromatosis,
fatty
of and
present in chondrocalcinosis
patient A liver
A
,
the and
bone
(Fig.
were showed
of
sclerosis
subchondral
glenoid
changes (L) knee
menisci
inferior
glenoid
pathic
pigment the
in
head
Osteoarthritic knees. The
the
and
cells
The
suggested.
further
work-up.
cirrhosis
abundant
idio-
with
mild
hemosiderin
and
in fibrous
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VOL.
No.
124,
FIG.
Case
4.
ii.
bilaterally.
cortex
Joint
i
and
Changes
in Idiopathic
(A and B) Degenerative This is characterized by
unusually
prominent
changes
Schumacher,” a with
he
described
of
both
joints,
in the 2nd and 3rd metacarpo-phalangeal joints narrowing, irregularity of the subchondral of the metacarpal heads. joint
osteophytes
joint
had
of
1964,
originally
associThe
prominent
changes
in
the
the et
a similar shoulders
and
with
elbows.
They stated that osteoarthritis best fit the clinical picture, although generalized joint involvement of the large joints was unusual. Hamilton et al.3 evaluated 32 patients
clinical
with
hemochromatosis.
evidence
of
arthritis
They in
i6.
found
In
others,
2
all
3 of
joint
symptoms
preceded
diagnosis, although of hemochromatosis of
Hamilton
in
i1.,’
case
In
ceded
M-P biopsy
and
patients,
the clinical major signs ent.
cases
2
involvement
Kra
in detail
de-
arthropathy
inflammation.
reported
space
these
hands, predominantly with minimal clinical
evidence
1965,
in distinctive hemochromatosis.
141
are noted
asymmetric
DISCUSSION
scribed ated
Hemochromatosis
et
the
joint
one of the was pressmptoms
the major signs al.’ accurately
pre-
of the disease. described the
as
roentgenographic findings in the hands narrowing of the M-P (primarily 2nd 3rd) joints, with erosion and segmentation
and
of
and
the
cst
underlying
chondrocalci menisci, of
bone,
formation.
Of nosis,
the
wrist,
ankle
In
i
joints
patient
i6
the
from
io
had
involving
the
triangular
symphysis were
sclerosis
patients
usually
although
and
and
the
pubis,
ligament hip
joints,
occasionally
involved.
this
histologic
group
Jack
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142
Fic. 5. Case space and joints.
examination clumps
in
These
had
pyrophosphate copy. The
on
by of
B)
63
thritis (o roentgenographic firmed.
of
Dymock patients.
per
polarized Hamilton
light
The
cent) and findings
incidence
of
the clinical were again
The has
high
caused
lationship
arand con-
viously al.3
of
arthropathy to
a
There seen
at
are narrowing the
incidence
inferior
3975
of the joint margin
of both
of chondrocalcinosis
speculation
between
position.
micros-
et al.3 was to include a
et al.2
concerning
the
re-
and calcium deAtkins a al.’ compared the prereported group by Hamilton a patients with hemochromatosis and chondrocalcinosis (I.H.)
group
chondrocalcinosis
calcification
g Fic.
MAY,
Both shoulders reveal identical abnormalities. sclerosis and cyst formation; osteophytes are
revealed crystals in rounded the synovial superficial layers. the characteristics of calcium study
extended total
(A and
ii.
subchondral
Twersky
of
iron
patients
(I.C.).
of fibrocartilage,
with Both
idiopathic groups
but
the
had
I.H.
I
6. Case ii. (A and B) Flattening bilaterally. Chondrocalcinosis
the medial both articular
of of
tibial plateau cartilage and
and subchondral menisci in seen
sclerosis are present in the (L) knee.
VOL.
124,
No.
group had cification Downloaded from www.ajronline.org by 149.56.172.34 on 06/21/16 from IP address 149.56.172.34. Copyright ARRS. For personal use only; all rights reserved
Joint
I
a much higher incidence of calof hyaline cartilage. The dis-
tribution
of
joint
different,
the
I.H.
ily
the
M-P
numerous
had
involvement
group
joints
and
involvement. analysis
of 19 patients
both
revealed
cartilage
but
changes
in multiple
the M-P joints. able from the
with
joints,
These changes
were seen
pseu-
role
has
of
structures
of iron
not
incorporation
cells
has
of
iron
well
the
patients
arthropathy
high
tients tosis; reports
with
joint
Past’
the
adjacent
in-
a
clinical
degenerative
in drinking
can by:
result
arthritis in hemochroma-
arthritis
pa-
subchondral
sclerosis
disof
water.4
In
idiopathic hemochromatosis iron is in the synovial fluid in the phagocytic synovial lining cells, and in the articular cartilage.’ However, no synovial inflammation is present. These findings suggest that iron has a direct deleterious effect on the articular cartilage, causing irreversible damage with resultant osteo-
Pyrophosphatase to soluble
hydrolizes
orthophosphate.
multiple
lucent
signs
of
idiopathic
hemochroma-
Department of Radiology State University of New York Downstate Medical Center 450 Clarkson Avenue Brooklyn, New York 11203 REFERENCES
(Kashin-Beck’s chronic ingestion
iron may activity
and
by
occurring
present
tilage.’ phosphate
hemoarthropathy
tosis.
associated
arthropathy
arthritis. Furthermore, pyrophosphatase enzyme
idiopathic
a distinctive
presented.
hemochromatosis;2”2
in Asiatic Russia ease), related to iron
of iron
incidence of with idiopathic of
distribution unexplained.
subchondral cysts, and chondrocalcinosis. The etiology of this arthropathy and its relationship to chondrocalcinosis are discussed. Occasionally, patients may present with this arthropathy before the onset of the
meta-
is corroborated
transfusion
with
and
classical
the
(3)
and
small
levels
(2)
bone
newly
blood
of
remains
pre-
The joint findings are characterized joint narrowing, prominent osteophytes,
in
high
pattern
can
of cartilage
are
and
of
pattern
The
plates
trabeculae
(I)
The involvement
Two
present
formed
same
SUMMARY
iron
physis. That
the
of calcium Iron
precipitation.
established.
studied.
were
chondrocalcinosis,
disorder
including
indistinguishin idiopathic
epiphyseal
of
with Martel’s6 with pseudogout cartilage degen-
secondary
cause
permit
this
into
been
may pyrophosphate
a primary
pyrophosphate
of joint
activity insoluble
in producing
been
amounts
in an
than
degeneration.
rats with an iron-dextran complex. hour after intravenous injection, iron noted in the hypertrophic cartilage
jected One was
with
rather
chromatosis
exact
arthritis
eration
sumably
hemochromatosis.7
The
enzyme
This is compatible that patients have a primary
crystals.
also
‘43
of
suggestion probably
hyaline and fibroand severe osteoar-
involvement,
thritic
also
primar-
wrists,
in
precipitation
joints,
primarily
dogout
was
involving
Hemochromatosis
decrease
while the I.C. group hip, knee and lumbar spine However, Martel and co-
large
workers’6
in Idiopathic
Changes
inhibit in carpyro-.
A
I. ATKINS, C. J., Mclvoa, J., SMITH, P. M., HAMILTON, E., and WILLIAMS, R. Chondrocalcinosis and arthropathy: studies in hemochromatosis and in idiopathic chondrocalcinosis. Quart. 7. Med., 1970, 39, 7 1-82. 2. DYMOCK, I. W., HAMILTON, E. B. D., LAWS, S. W., and WILLIAMS, R. Arthropathy of hemochromatosis. Ann. Rheumat. Dis., 1970, 29,
469477.
3. HAMILTON, E., WILLIAMS, R., BARLOW, K. A., and SMITH, P. M. Arthropathy of idiopathic hemochromatosis. Quart. 7. Med., 1968, 37, 171-182.
4. HOLLANDER, J. L. Arthritis and Allied Conditions. Lea & Febiger, Philadelphia, 1966. 5. KRA, S. J., HOLLINGSWORTH, J. W., and FINCH, S. C. Arthritis with synovial iron deposition in patient with hemochromatosis. New England 7. Med., 1965, 272, 1268-1271.
Jack
‘44
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6.
Twersky
W., CHAMPION, C. K., THOMPSON, G. R., and CARTER, T. L. Roentgenologically distinctive arthropathy in some patients with
MARTEL,
pseudogout
RAD.
1HERAPY
syndrome. AM. & NUCLEAR
J.
MED.,
3970,
MARTEL,
ology. Chicago, MUIRDEN,
hemochromatosis. RAD.
1970,
1972,
K. D.,
p.
arthritis. 12.
SELLA,
7.
and
NUCLEAR
MED.,
H. R.,
JR. Hemochromatosis and Rheum., 1964, 7, 41-50. E. S., and GOODMAN, A. H. Arthropathy
Arth.
secondary
103.
J. ROENT-
AM.
&
THERAPY
575-580.
109,
II. SCHUMACHER,
W. Comment. In: Yearbook of RadiYearbook Medical Publishers, Inc.,
SENATOR, G. B. Iron in synovial membrane in rheumatoid arthritis and other joint diseases. Ann. Rheumat. Dii., 1968, 27, 38-48. 9. PAST, W. L. Histologic demonstration of iron in 8.
idiopathic
109,
3975
osseous tissue. Am. 7. Path., 1961,39, 443-453. Ross, P., and WooD, B. Osteoarthropathy in GENOL.,
ROENTGENOL.,
587-605.
7.
10.
MAY,
Bone
to
&
transfusion
Joint
Surg.,
hemochromatosis. 1973,
55-A,
1077-
io8i. 13.
WARDLE,
joint Rheumat.
E. N., changes Dii.,
and in 1969,
PATTON, J. T. hemochromatosis. 28,
15-23.
Bone
and Ann.