Musculoskeletal manifestations of paget's disease of bone.

1121
MUSCULOSKELETAL MANIFESTATIONS OF
PAGET’S DISEASE OF BONE
ROY D. ALTMAN with the clinical assistance of BARBARA COLLINS
A review of 290 patients with Paget’s disease of
bone revealed 83% had one or more rheumatic syndromes. The most common finding was back pain (37%)’
most often related to an independent osteoarthritic
process or Paget’s disease precipitating or complicating
spinal osteoarthritis. Paget’s disease as a sole source of
back pain was distinctly uncommon (2%).Osteoarthritis
related to Paget’s disease was present in the hip (30%)
and knee (11%). Paget’s disease in spondylitis was present in 4 patients and in ankylosing hyperostosis in 4 patients. The latter group had very active Paget’s disease.
Rheumatoid arthritis (1%)’ hyperuricemia (20%)’ and
gout (4%)did not appear increased in this group.
In 1876 Sir James Paget reported “On a Form of
Chronic Inflammation of Bones (Osteitis Deformans)”
(1). His 5 patients had radicular lower extremity pain,
long bone pain, or both. The original patient had a
single episode of a synovial effusion of the knee. When
the patient died, a pathologic specimen of the femur
demonstrated proximal and distal femoral Paget’s disease with femoral head remodeling and osteophyte formation. Paget further stated “In its earlier periods, and
sometimes through all its course, the disease is attended
From the Arthritis Division, University of Miami, and the
Arthritis Section, Miami Veterans Administration Hospital, Miami,
Florida.
Supported in part by the Clinical Research Unit RR 00261
General Clinical Research Centers Program of Research Sources
NIH.
Roy D. Altman, MD: Associate Professor of Medicine, Division of Arthritis, University of Miami, Chief, Arthritis Division, Miami Veterans Administration Hospital; Barbara Collins, ARNP.
Address reprint requests to Roy D. Altman, MD, University
of Miami School of Medicine, P.O. Box 016960, Miami, Florida
33101.
Arthritis and Rheumatism, Vol. 23, No. 10 (October 1980)
with pains in the affected bones, pains widely various in
severity and variously described as rheumatic, gouty, or
neuralgic, nor especially nocturnal or periodical.” His
second report, 6 years later, included 7 cases (2): 1 was
asymptomatic, but the remaining 6 had various rheumatic complaints including ankle pain related to a
bowed tibia, back pain, knee pain, gout, and interphalangeal osteoarthritis.
In 1936 in a review of 116 cases of Paget’s disease, Gutman and Kasabach reported that pain was
present in 77% of their patients, particularly in the leg,
lower back, or hips (3).
Several reports have related Paget’s disease and
osteoarthritis of the hip (4-6) and knee (7). However,
not all reports agree with the relationship of Paget’s disease to osteoarthritis (8-11). In a careful review of 55
patients, Franck and coworkers emphasized the common relationship of rheumatic diseases to severely afflicted patients with Paget’s disease (7), documenting
many of the clinical manifestations suggested by Sir
James Paget.
This report is an analysis of 290 patients referred
for evaluation of their Paget’s disease. Rheumatic syndromes and Paget’s disease were correlated with clinical, laboratory, roentgenographic, and bone scan parameters.
PATIENTS AND METHODS
Two-hundred ninety patients were evaluated for Paget’s disease of bone over the period from 1971 to 1980. Characteristics of the population are listed in Table 1.
Patients were evaluated with special reference to arthritic and Paget’s disease signs and symptoms. Examination
was directed at special problems that may relate Paget’s disease and arthritis, such as abnormal gait and stance, need for
ALTMAN
1122
Table 1. Paget’s disease: characteristics of 290 patients
Age
Sex
Race
Duration of chief complaint
Duration of diagnosis of Paget’s
disease
Family history of Paget’s
disease
Renal lithiasis
Fractures of pagetic bone
Rate
Alkaline phosphatase (N<l)*
Hydroxyprolenet
67.4 f 8.7 SD years; range 36-91
1.3 men: I woman
286 white: 4 black
6.2 f 7.3 years; range 0-50
9.6 f 9.6 years; range 0-47
42
24
46
1.6/100 patient years
4.7 f 4.7 range 0.4-68.8
228 f 296 mg/24 hr range 4-1486
* Value expressed as finding divided by upper limit of normal range.
t Normal range 9-38
mg/24 hr.
assisted ambulation, long bone deformity, bone tenderness,
functionally short extremity, and range of motion of articulations adjacent to pagetic bones.
Laboratory values reviewed included serum alkaline
phosphatase in 286 patients, urine hydroxyproline excretion
in 130 patients, and serum uric acid in 186 patients. Serum alkaline phosphatase was performed by an automated colorimetric method at the University Medical Center, utilizing a
thymolphthalein monophosphate substrate (12). Over the period of the study, there were several changes in the values for
the normal range. For this reason, alkaline phosphatase is reported as a multiple of the upper limits of the normal range
during the period of collection, i.e. normal <1; elevation > l .
These values were recorded prior to pagetic suppressive therapy. Urine hydroxyproline was determined by the modified
method of Kivirikko (13). Serum uric acid was performed by
an automated colorimetric uricase method in which normal
values are less than 8 for men or women (14,15).
Total body x-rays were reviewed in 104 patients.
X-rays of involved areas of Paget’s disease and/or arthritis
were taken in the remainder of the patients. Bone scans were
performed in 225, and computer assisted or axial tomography
of the spine was reviewed in 34 patients.
Narrowing of the radiologic joint space on hip roentgenograms was defined as medial, superior, or superior and
medial. The medial refers to a nearly focal defect in the superior medial aspect of the joint just superior to the ligamentum
teres. Superior narrowing and superior and medial narrowing
correlate respectively to the “superior migration” and “superomedial migration” described by Resnick (16).
present in 11%, and pain about the skull often related to
vertigo was the primary complaint in 10%. Seven patients had decreased auditory acuity and only 8 patients
were seen with no symptoms referrable to the diagnosis
of Paget’s disease. The presenting complaint (e.g. pain
or decreased hearing) initiated the workup leading to a
diagnosis of Paget’s disease in 41% of patients. The remaining patients were diagnosed by x-ray for other purposes (29%), by pursuit of an abnormal alkaline phosphatase value (16%), occasionally by spontaneous
fracture through pagetic bone, by clinically apparent
skull or long bone deformity, or because a relative had
Paget’s disease.
Rheumatologic examination revealed signs suggestive of osteoarthritis of one or more joints in 265 patients; e.g. 91% of the patients had crepitus or decreased
range of motion of one or more joints. The joints most
commonly involved were the cervical spine, shoulder,
hip, and knee.
Clinical findings suggestive of Paget’s disease
were present in 208 (72%). Patients had one or more
findings of skull deformity with or without scalp vein
distention, pagetic stature or gait, angioid streaks on retinal examination, high output cardiac state, compromised pulmonary reserve, or long bone deformity
with or without increased palpable temperature over the
involved extremity. Of patients with clinical findings of
Paget’s disease, only one such finding above was present
in 20%, and five or more of these findings were evident
in 11% of patients with an average of 1.8 findings overall.
In patients with skull involvement on examination, extensive Paget’s disease was present as exemplified by abnormal “Pagetic stance” in 50%, long
bone deformity in 40%, headache in 52%, hearing loss in
46%, dilated scalp veins in 45%, high output cardiac
state in 28%, and decreased range of motion of the neck
in 75%.
Table 2. Paget’s disease: reason for consultation
RESULTS
Referral was for management of Paget’s disease
in 252 patients. However, all but 15 patients had pain
that precipitated their consultation (Table 2). Back pain
was most common, being the major complaint in 34%,
and a secondary complaint in an additional 8 patients
(37% total). Back pain was nonspecific and often radicular. Pain around the hip, aggravated by ambulation and
relieved by sitting or reclining, was present in 22%.
Bone pain associated with long bones or pelvis was
Back pain
Hip pain
Bone pain
Headache
Knee pain
Decreased hearing
Neck pain
No symptoms
Other
Total
Number of
patients (%)
Duration years
(mean f SD)
98 (34)
65 (22)
33 (11)
29 (10)
19 (6)
7 (2)
5 (2)
8 (3)
27 (9)
7.8 f 9.5
5.0 f 6.0
4.0 f 5.1
7.8 f 6.5
5.4 f 5.5
9.1 -t 9.7
4.0 f 5.6
1.0 f 1.6
6.5 f 7.9
290 (100)
6.2 f 7.6
MUSCULOSKELETAL MANIFESTATIONS
About half of the patients who exhibited a pagetic stance had extensive disease with skull enlargement, abnormal gait, and long bone deformities. Half
the patients with hearing loss had skull deformity, and
half the patients with skull deformity had hearing loss.
An abnormal “pagetic stance” was present in 38 (13%)
patients who had the waddling gait characteristic of this
malady. Abnormal gait related to long bone deformity
was present in an additional 25 (9%) patients without
pagetic stance.
The male predominance was accentuated for
those with pelvic Paget’s disease (3.2 men: 1 woman)
and reversed in patients with platybasia (1 man: 1.4
women).
The mean serum alkaline phosphatase was 4.7 &
4.7 SD times the upper limits of the normal range. A
normal alkaline phosphatase value was present in only
12 patients (4.4%). These patients had mostly monostotic disease; however, a few were symptomatic from
the involved bones; e.g. calcaneal or navicular foot pain
in 2 patients. Table 3 relates the diagnosis of arthritic
and pagetic induced pain to serum alkaline phosphatase. The highest elevations of the alkaline phosphatase
were present in patients who had pain related to pagetic
and arthritic changes about the spine, pagetic changes
Table 3. Rheumatic diagnoses and pain syndromes
Patients
No.
(%)
Alkaline
phosphatase
(mean f SD)
50
88
31
18
30
11
4.4 f 2.9
6.6 f 8.6
7.0 f 12.6
45
54
7
4
15
16
19
2
1
5
3.2 f 2.8
10.9 f 12.1
4.4 f 4.2
2.6 f 2.4
7.2 f 5.8
4
1
21.4 f 31.9
4
11
4
13
24
45
8
1
4
1
4
16
3
1.8 f 0.5
8.2 f 9.1
1.5 f 0.5
2.6 f 0.5
5.1 f 4 . 9
4.8 f 4.5
5.1 f 4.9
41
19
33
4
14
7
I1
1
7.2 f 4.6
11.7 f 7.3
6.6 f 12.4
7.8 f 5.0
Lower extremity
Long bone pain
Hip: DJD* and Paget’s disease
Knee: DJD and Paget’s disease
Axial skeleton
Spine: DJD
Spine: DJD and Paget’s disease
Spine: Paget’sdisease
Spondylitis
Ankylosing hyperostosis
Ankylosing hyperostosis
with Paget’s disease
Other rheumatic syndromes
Rheumatoid arthritis
Gout
Chondrocalcinosis
Fibrosing syndromes
Nonarticular rheumatism
InterphalangealDJD
Miscellaneous
Other pain syndromes
Diffuse head pain
Platybasia
Pelvic pain
Miscellaneous
~~~
* DJD
= degenerative joint disease.
8
1123
of ankylosing hyperostosis, and skull involvement severe enough to cause platybasia.
Bone pain with or without pain from other
sources was considered to be present in 135 patients
(47%).Bone pain was rarely nocturnal. It was often dull,
aching, and aggravated by activities such as weight
bearing. Actual bone tenderness was present in only 20
patients. Pain related to platybasia was often localized
to the neck and occiput. When this pain was associated
with decreased range of motion of the neck, it was difficult to differentiate from cervical osteoarthritis. Pelvic
bone pain was particularly difficult to separate from primary or secondary osteoarthritic changes of the hip. A
clear definition of the origin of pain was even more difficult in this group when there were abnormalities of the
lumbosacral spine.
Urine hydroxyproline excretion prior to therapy
for Paget’s disease was normal in 10 patients. All 10 had
normal serum alkaline phosphatase, as discussed. Degree of hydroxyproline elevation was not dependent
upon diagnosis.
Lower extremity
Long bone deformity was present in 45% of patients. Many of these patients described low back, hip,
knee, or ankle pain related to activities. Examination often demonstrated an anterior and lateral bow to the
thigh or leg, causing a functional flexion contracture at
the knee often in spite of extension at the knee of 0 to
- 10”. Increased temperature estimated by palpation of
a pagetic limb, particularly the tibia, was present in
two-thirds of those patients with long bone deformity.
Synovitis and/or crepitus was present in 8 1% of patients
with pagetic deformity around the knee, whereas these
changes were present in 51% of the patients without juxtaarticular Paget’s disease of the knee.
When present, long bone pain was frequently aggravated by activities, i.e. weight bearing. This made the
differentiation of long bone pain from arthritis of the
hip or knee more difficult unless pain could be localized
over a specific area of the long bone. This differentiation was facilitated when pain localized over the site of
a radiologic osteolytic wedge. A functionally short extremity was present in 12 patients with bowing of long
bones.
Pain in one or both hips was present in 88 patients (Table 4). Pain was described in the medial thigh
as well as radiating from the trochanteric region. Bone
pain from the pelvis or proximal femora was often difficult to differentiate from arthritic pain from the hip.
ALTMAN
1124
Table 4. Hip pain and pelvic x-ray changes
Number of patients with
X-ray of pelvis
Pain
N o pain
Not pagetic -joint space normal
Joint space narrow
(superior)
Pagetic-joint space normal
Joint space narrow
Superior
Medial
Superior and medial
0
91
6
4
78
3
6
63
30
0
18
57
26
Total
88
I90
4
This was occasionally facilitated by arthrocentesis of the
hip with intrasynovial lidocaine anesthesia.
Pain was interpreted to be from osteoarthritis of
the hip in 4 patients in whom the hip joint was radiographically normal. Pain was present in 70% of patients
with an abnormal roentgenographic joint space. Normal range of motion of the hip was present in 60% of
patients with decreased roentgenographic joint space.
Full range of motion of the hip was present in 12 patients with pain on exercise or motion of the hip and decreased radiologic joint space. Most radiographic narrowing involved both the medial and superior aspects of
the joint space. However, 22 patients had medial joint
narrowing with normal superior aspects of the hip joint.
One x-ray demonstrated a giant acetabular cyst of the
medial ileum and ileopectineal region. Osteophyte formation was present in 40 patients, and 6 patients had
protrusio acetabuli. Arthritic changes in the hip by xray were present in 32% of patients with long bone deformity. In 15 patients, there was no adjacent pelvic pagetic change. These patients had superior joint space
narrowing. Even in the presence of pelvic Paget’s disease, the bone scan was useful in evaluating the extent
of osteoarthritic involvement of the hip. An anterior
camera spot view of the hip revealed the following: 1)
decreased uptake in the region of the femoral neck indicative of reduced vascular supply to the femoral head,
2) femoral head subchondral bone necrosis with increased uptake of the nuclide that localized to the area
of necrosis; this was differentiated from the pelvic uptake in the absence of femoral head Paget’s disease, 3) a
diffuse uptake around the hip which suggested a synovitis of the hip in the absence of femoral head Paget’s
disease.
Examination suggesting osteoarthritis of the
knee was present in 144 patients. This was related to
distal femoral or proximal tibia1 Paget’s in 31 (22%) patients. In those with pagetic changes around the knee,
knee pain was dull and aching, aggravated by activity,
and associated with considerable stiffness. Range of motion demonstrated full extension in 16, hyperextension
(0 to -10’) in 8, and flexion contractures (+lo” or
more) in 7. Incomplete flexion (up to 1 10’) was present
in 16. Osteophytes were present in 28 patients and radiologic joint space narrowing was present in all. Knees
were stable in anteroposterior and mediolateral planes
even if there had been prior medial meniscectomy (4
patients). There was frequent bony deformity such as an
enlarged patella, enlarged condyle, or enlarged proximal tibia. Arthroscopy performed on 1 patient with distal femoral pagetic changes revealed an inflammatory
synovitis with yellowing of cartilage and deep condylar
cartilage fissures without fraying or ulceration. Synovial
effusions of the knee were aspirated in 9 patients. Synovianalysis revealed recurrent Type I effusions by criteria
of Ropes and Bauer. Microscopically they contained
few mononuclear cells and numerous fibrin strands. Synovial effusions remitted in 3 patients after successful
chemical suppression of their Paget’s disease. Of patients with knee arthritis and juxtaarticular Paget’s disease, long bone deformity was present in 79%. Assisted
ambulation (cane) was needed in 36% of patients with
Paget’s disease and related knee arthritis; however, a 76year-old patient with severe distal femoral Paget’s disease related to recurrent knee effusions, knee flexion
contractures, and severe pain was able to play handball
almost daily.
Spine
Lumbar spine pain with some evidence of lumbosacral Paget’s disease was the chief complaint in 98
patients (34%) and was significant in an additional 8 patients (37% total). Back pain was nonspecific, often with
radiation into both gluteal regions and often into the
thighs, legs, or feet. A history suggestive of the cauda
equina syndrome was present in 23 patients.
Physical findings were often nonspecific, but 38
patients had a typical pagetic stance with straightened
lumbar spine, hips, and knees flexed, and paralumbar
muscle spasm. Only 49 patients (46%) had physical
findings of a positive sciatic stretch sign.
If the spine were only partially involved with Paget’s disease, the bone scan could outline pagetic involvement of the neural arch. Routine lumbosacral
spine x-rays with obliques did not always define Paget’s
disease in the neural arch. Spinal articular facets were
only partially defined because many of the patients had
osteopenia with minimal apparent sclerosis. Lumbar
disc narrowing was present in 36 patients and seemed
related to symptoms in 5.
Computer assisted or transaxial tomography of
1125
MUSCULOSKELETAL MANIFESTATIONS
the lumbar spine was obtained in 34 patients. These
techniques helped define arthritic and/or pagetic involvement of the neural arch by accentuating the coarse
abnormal trabeculations of Paget’s disease and by defining the size and shape of the articular facets, neural
canal, and lateral recesses (medial aspect of the intervertebral foramina).
Back pain was considered to be related to solely
pagetic changes in the spine in only 7 patients. Lumbar
back pain was thought to be related to pagetic combined with arthritic changes in 45 patients where there
was distortion of the articular facets and evidence of
juxtaposed pagetic bone. Of patients with lumbar pain,
45 (46%) had evidence of arthritis or discogenic disease
that did not seem related to the pagetic process.
Of patients with pain severe enough to warrant
transaxial or computer assisted tomography, abnormalities were moderately severe: 16 had spinal stenosis of at
least one level, 8 had a lateral recess syndrome, and 10
had both of these findings.
Dorsal spine pain was present in 6 patients with
postmenopausal osteopenia related to compression fractures of seemingly nonpagetic bone.
One patient had an enlarged dorsal vertebra with
spastic paraparesis related to spinal impingement documented by myelography.
Marie-Strumpell’s ankylosing spondylitis was
present clinically and radiographically in 2 patients.
Both had pagetic changes of the spine with pagetic involvement of syndesmophytes. Both had extensive syndesmophyte formation and minimal motion of the spine
by examination. Immobility was more significant than
pain in both patients.
Psoriatic spondylitis was present in 2 of 14 patients with psoriasis. Both patients had pagetic involvement of their lumbar and dorsal spine as well as
syndesmophytes. In these 2 patients, immobility was
also more significant than pain. No other forms of psoriatic associated arthritis were present in these patients.
Ankylosing hyperostosis (diffuse idiopathic skeletal hyperostosis) was recognized in 19 patients. Four of
these patients had pagetic changes of the anterior and
lateral spinal calcifications. None were symptomatic,
but 3 of 4 had significant elevations of their alkaline
phosphatase. Dorsal stiffness was present but not clinically significant.
Other rheumatic syndromes
A history of rheumatoid arthritis was present in 3
patients, but none had active synovitis nor deformity.
Two had received chrysotherapy in the past. Rheuma-
Table 5. Paget’s disease: serum uric acid of 149 patients
~
Serum uric acid
No. of patients
Percent
Normal range
Increase 1 mg%
Increase 1-2 mg%
Increase 2-3 mg%
Increase 3-4 mg%
118
20
8
I
2
19
13
5
1
I
toid factor was absent in 2 patients and titered 1 : 80 in 1
patient. An additional patient had Sjogren’s syndrome
with mildly active bilateral synovitis of small jointsthis patient was interpreted to have a rheumatoid syndrome associated with Sjogren’s syndrome.
A history of gouty arthritis was present in 11 patients. The serum uric acid of patients not on allupurinal is reflected in Table 5. The incidence of hyperuricemia was 20%. Allopurinal was being taken by 24
patients, 4 with a history of gout, and 6 with a history of
renal lithiasis. Only one patient had prior identification
of uric acid in the synovial fluid; this patient had multiple tophaceous deposits, the only patient with tophaceous gout.
Chondrocalcinosis was noted in only 4 patients.
No attacks of pseudogout were reported nor recognized.
Bursa1 calcification was similarly uncommon, being
noted in only 5 patients.
Fibrosing syndromes of Dupuytren’s contracture
(10 patients-4%) and Peyronie’s disease (4 patients)
were noted, but did not seem to relate to the activity of
their Paget’s disease. Knuckle pads were present in only
1 patient with Dupuytren’s contracture. An age and sex
matched population of 170 patients demonstrated 7 patients with Dupuytren’s contracture (4%). A control
population was not available for Peytronie’s disease.
Several patients had various forms of nonarticular rheumatism. This included periarthritis (8 patients), carpal tunnel syndrome (5 patients), tendonitis
(5 patients), fibrositis (3 patients), epicondylitis (2 patients), and meralgia paresthetica (1 patient). These
findings seemed to be independent of their Paget’s disease in most cases. Two patients had periarthritis of the
shoulder with scapular or proximal humoral Paget’s disease.
Znterphalangeal osteoarthritis was present in 45
patients. This group included 40 women and 5 men and
the disease was variable in its manifestations. There was
no correlation to the pagetic process.
Upper extremity long bone deformity produced
special problems. Two patients with humoral bowing
were unable to feed or shave themselves with the right
hand due to functional extensor contractures related to
the deformity. A single patient with marked bowing of
ALTMAN
1126
the radius ruptured the fourth and fifth extensor tendors
of the extensor digitorium longus, reminiscent of wrist
disease in rheumatoid arthritis.
Obliteration of the sacroiliac joints was frequently associated with combined sacral and iliac Paget’s disease. No patient seemed to have symptomatic
sacroiliitis.
DISCUSSION
In this review, 242 of 290 (83%) patients referred
for evaluation of their Paget’s disease had pain referrable to the musculoskeletal system. There were 396
diagnoses of rheumatic disease or bone pain origin.
Pain is a recognized problem in Paget’s disease; in the
past, it was classified as neuritic or bony. There have
been few reviews emphasizing the rheumatic associations of Paget’s disease.
These patients had moderately severe Paget’s
disease, based on the fact that they have been referred
for evaluation, had moderate or marked elevation of
serum alkaline phosphatase and/or urine hydroxyproline, and 97% were symptomatic. For these reasons,
our comments on the relation of rheumatic complaints
to Paget’s disease should be interpreted in the context of
the symptomatic patient with Paget’s disease.
Sir James Paget’s original patient had a remodeled femoral head (1). Since that time, there has been
question concerning any general relationship between
Paget’s disease and osteoarthritis (8- 1 1). Recent radiologic reviews indicate that the overall incidence of
osteoarthritis in Paget’s disease may not be increased
(10, 1 1); however, the incidence of moderately advanced
hip joint narrowing in 43% of our patients seems quite
high. As in prior reports (6, lo), there was diffuse medial
and superior joint narrowing in the majority of patients,
but medial joint narrowing and protrusio acetabuli
seemed common.
Certainly not all juxtaarticular Paget’s disease
was related to osteoarthritic change, but certain factors
are believed to influence the normal dynamic and anatomic architecture of the joint. These are:
1 . Juxtaarticular bony enlargement: Pagetic subchondral bone slowly enlarges, similar to pagetic subperiosteal enlargement. Interestingly, cartilage appears
to adapt and expand to cover the enlarged subchondral
structure. When the normal incongruity of the articular
surface is altered, secondary cartilage degeneration may
occur (17). Examples of this may be an enlarged femoral head due to Paget’s disease, and enlargement of the
ileopectineal line providing medial joint impingement
on the femoral head.
2. Altered biodynamics: Bowing and lengthening
of lower extremities alter gait and may well alter
biodynamic pressure points of juxtaposed cartilage
structures without the hip or knee joints (18). A similar
process may occur in the spinal apophyseal joints due to
localized enlarged pagetic vertebral bone as well as enlarged vertebrae that alter spinal dynamics.
3. Altered subchondral support: Enlargement of
subchondral pagetic bone is not necessarily uniform.
Uneven expansion provides an uneven base of cartilage
support (19). The need for adequate subchondral support has recently been emphasized (20). This uneven
support to the cartilage may precipitate osteoarthritic
degeneration.
The value of radionuclide scanning in rheumatology has recently been reviewed (2 1,22). The presence
of pagetic uptake of the nuclide does not obviate the
value of bone scanning as discussed.
Back pain was the single most common complaint in this series of patients. Paget’s disease, in the
author’s opinion, has been given more emphasis than it
deserves as cause of back pain (9,23,24). A careful evaluation of the cause of back pain appears appropriate
since primary and secondary osteoarthritic changes often complicate the evaluation of pain. In this study,
only 7% of patients with back pain were considered to
have solely Paget’s disease as the source of their pain.
Our findings confirm a prior report of the interesting finding of Paget’s disease in ankylosing spondylitis (10). However, a true increase in Paget’s disease of
the spine in ankylosing spondylitis or psoriatic associated spondylitis cannot be clearly concluded from such
small numbers of patients.
Another interesting finding was the pagetic
changes in the spinal calcifications of ankylosing hyperostosis. This was associated with extensive and severe
Paget’s disease in 3 of the 4 cases, but was not in itself
symptomatic.
The occurrence of gout, hyperuricemia, chondrocalcinosis, and rheumatoid arthritis did not seem high,
and conflicts somewhat with prior reports (7,25,26). Our
findings are consistent with reports of hyperuricemia in
the population of the United States (27,28) and
chondrocalcinosis in Paget’s disease (29).
Dupuytren’s contracture was an interesting finding that did not exceed the incidence in our own population and was even less than that reported in certain
population studies (30). However, the finding of Peyronie’s disease in 4 patients may seem high, but no control population was available for comparison.
MUSCULOSKELETAL MANIFESTATIONS
ACKNOWLEDGMENTS
The author is indebted for the clinical assistance of
Fred Gargano, MD, Mark Brown, MD, Evelyn Altman, Oscar Gans, to Daphne Stoddart for the preparation of the manuscript, and to David s. Howell, MD for his review of this
manuscript.
REFERENCES
I . Paget J: On a form of chronic inflammation of bones (osteitis deformans). Med Chir Trans 60:37, 1877
2. Paget J: Additional cases of osteitis deformans. Med Chir
Trans 65:225, 1882
3. Gutman AB, Kasabach H: Paget’s disease (osteitis deformans). Am J Med Sci 191:361, 1936
4. Machtey I, Rodnan GP, Benedek TG: Paget’s disease of
the hip joint. Am J Med Sci 251524, 1966
5. Roper BA: Paget’s disease involving the hip joint-a
classification. Clin Orthop Re1 Res 80:33, 1971
6. Goldman AB, Bullough P, Kammerman S, Ambos M:
Osteitis deformans of the hip joint. Am J Roentgenol
128:601, 1977
7. Franck WA, Bress NM, Singer FR, Krane SM: Rheumatic manifestations of Paget’s disease of bone. Am J
Med 56592, 1974
8. Jaffe HL: The classic Paget’s disease of bone. Clin Orthop
Re1 Res 127:4, 1977
9. Milgram JW: Radiographical and pathological assessment of the activity of Paget’s disease of bone. Clin Orthop Re1 Res 127:43, 1977
10. Guyer PB, Dewbury KC: The hip joint in Paget’s disease
(Paget’s “coxopathy”). Br J Radio1 51:574, 1978
11. Guyer PB: The clinical relevance of radiologically revealed Paget’s disease of bone (osteitis deformans). Br J
Surg 66:438, 1979
12. Roy AV: Rapid method for determining alkaline phosphatase activity in serum with thymolphthalein monophosphate. Clin Chem 16(5):431, 1970
13. Kivirikko KI, Laitinen 0, Prockop DJ: Modifications of a
specific assay for hydroxyproline in urine. Anal Biochem
19:249, 1967
14. Praetorius E, Poulsen H: Enzymatic determination of uric
acid with detailed directions. Scand J Clin Lab Invest
3:273, 1953
1127
15. Trinder P: Determination of glucose in blood using glucose oxidase with an alternative oxygen acceptor. Ann
Clin Biochem 6:24, 1969
16. Resnick D: Patterns of migration of the femoral head in
osteoarthritis of the hip-roentgenographic-pathologic
correlation and comparision with rheumatoid arthritis.
Am J Roentgenol Radium Ther Nucl Med 124:62, 1975
17. Walmsley T: The articular mechanism of the diarthroses.
J Bone Joint Surg 10:40, 1928
18. Kaye CF, Lippiello L, Mankin H, Numata T: Evidence
for a pressure sensitive stimulus receptor system in articular cartilage. 26th Annual Orthopedic Research Society,
Atlanta, Georgia, February 5-7, 1980
19. Freund E: Zur Frage der Ostitis deformans Paget. Virchows Archiv 274:1, 1929
20. Radin EL, Paul IL: Importance of bone in sparing articular cartilage from impact. Clin Orthop Rehab Res 78:342,
197 1
21. Christensen SB, Arnoldi CC: Distribution of 99”’Tc-phosphate compounds in osteoarthritic femoral heads. J Bone
Joint Surg 62-A( 1):90, 1980
22. Greyson ND: Radionuclide bone and joint imaging in
rheumatology. Bull Rheum Dis 30(7):1034, 1979- 1980
23. Barry HC: Paget’s disease of bone. Edinburgh, E. & S.
Livingstone Ltd, 1969, p 33
24. Altman RD, Brown MD, Gargano FP: Transaxial tomography in the assessment of back pain. Arthritis Rheum
21(5):541, 1978
25. Franck WA, Bress NM, Singer FR, Krane SM: Hyperuricemia and gout in Paget’s disease of bone. Arthritis
Rheum 17(4):496, 1974
26. Talbott JH, Yii T: Gout and uric acid metabolism. Statton Intercontinental Medical Book Corporation, 1976
27. Mikkelsen WM, Dodge HJ, Valkenburg H: The distribution of serum uric acid values in a population unselected
as to gout or hyperuricemia. Am J Med 39:242, 1965
28. Hall AP, Barry PE, Dawber TR, McNamara PM: Epidemiology of gout and hyperuricemia. Am J Med 42:27,
1967
29. Boussina I, Gerster J-C, Epiney J, Fallet GH: A study of
the incidence of articular chondrocalcinosis in Paget’s disease of bone. Scand J Rheumatol 5:33, 1976
30. Early PF: Population studies in Dupuytren’s contracture.
J Bone Joint Surg 44B:602, 1962
DISCUSSION
Dr. Johnston: Have you seen new sites develop during
in
your followup? We have Seen new lesions
ribs, even in patients undergoing therapy.
Dr. Altman: We have not noted new lesions even with
sequential bone scans. We have used bone scans for
following therapy of symptomatic lesions. The bone
scan may be the only parameter that changes in the
patient with a normal or near normal alkaline phos-
phatase such as the calcaneus in one of my patients.
Dr. Bijvo& We have found that at times it may take
longer to see change in the bone scan than in the alkaline phosphatase values.
Dr. Singer: In our patients the Gallium scan has provided a more sensitive parameter of pagetic bone activity than the phosphate scanning agents.