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.

Joint changes in idiopathic hemochromatosis.

Two patients with idiopathic hemochromatosis and a distinctive arthropathy are presented. The joint findings are characterized by joint narrowing, pro...
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