Joint Program in Nuclear Medicine
Hypertrophic Osteoarthropathy
Martin Charron, MD
J. Anthony Parker, MD, PhD
December 1, 1987
Presentation
A 47 year-old white male presented with coughing for three months,
accompanied
by painful swollen knees, legs and ankles.
Imaging Findings
A chest X-ray (shown by arrow) and a
CT scan (shown by arrow) done 1 month after
presentation showed a 4 cm right lower lobe mass. The lung washing
was positive for bronchial adenocarcinoma of the lung. The
bone scintigram revealed increased uptake in the lower
extremities consistent with hypertrophic pulmonary osteoarthropathy
(HPO). He
was started on PFM (Platinium, 5-Fluorouracil, Methotrexate).
Discussion
Clinical:
Secondary hypertrophic osteoarthropathy (HO), also known as the
Marie-Bamberg
Syndrome, consists of clubbing of the finger and toes, periostitis
of the long
bones, sometimes a polyarthritis resembling rheumatoid arthritis,
hyperhydrosis, flushing, and blanching. Ossifying periostitis
is seen on x-ray
at the distal end of the shaft of the long bones as an opaque
line of new bone
formation, separated from the underlying cortex by a narrow radiolucent
band.
As it accumulates, the periosteal new bone becomes irregular,
rough and
undulating and eventually fusing with the cortex. The radiological
differential diagnosis includes thyroid acropachy, chronic venous
stasis,
hypervitminosis A and infantile cortical hyperostosis.
Four to twelve percent of the patients with bronchial carcinoma
develop hypertrophic osteoarthropathy. But
HO may precede the discovery of the neoplasm by several months.
There are over
60 extra and intra-thoracic diseases which have been associated
with hypertrophic osteoarthropathy. When
there is no know associated disease this condition is referred
as
pachydermoperiostitis, a rare familial disorder.
Pathophysiology:
Histologically there is an inflammatory reaction
with round cell infiltrates and latter the periosteal new bone
formation
develops. The pathogenesis of hypertrophic osteoarthropathy remains elusive. Estrogens,
circulating
factors, neurogenic factors and growth hormone have been postulated
to play a
role (5). Of special interest is the regression of the new periosteal
bone which
frequently occurs after removal of a lung carcinoma. The pre-operative
pain
may also regress completely in 4-5 days (3).
Thoracotomy, hilar neurectomy, vagotomy, ipsilateral occlusion
of the pulmonary
artery, radiotherapy, chemotherapy, intercostal nerve section,
laparatomy (1),
and chemical vagatomy sometimes lead to remission of the symptom
(2), but the
mechanism by which these diverse procedures lead to remission
is uncertain.
Nuclear Medicine:
Radionuclide bone imaging is a very sensitive method for detecting
abnormalities of hypertrophic osteoarthropathy. A diffuse symmetric increased uptake in
the diaphysis and
metaphysis of tubular bones along their cortical margin create
a distinctive
"parallel track". Associated synovitis can lead to increased
radionuclide
uptake in the periarticular region. Ali (1) reported the following
distribution of bony involvement in 48 patients:
- tibia and fibula 95%%,
- femur 88%%,
- hands and carpels 88%%,
- radius and ulna 85%%,
- feet 81%%,
- scapula 67%%,
- mandible 42%%,
- clavicle 33%%,
- ribs 2%%, and
- pelvis 2%%.
Of note, none
of these 48
patients had increased spinal uptake. A patchy uptake in the
long bones is not unusual.
These scintigraphic findings frequently appear before roentgenographic
findings, correspond well with clinical manifestation and decreased
after treatment.
References
1) Ali, et al. Distribution of HPO. AJR 1980; 134:771-780.
2) Resnick, et al. Diagnosis of bone... Saunders 1980; 3:2988.
3) Rosenthal, et al. Skeletal imaging. ACC 1984; 297.
4) McFee. SNM 1987; 17(4):346.
5) Galasko. Radionuclide scintigraphy in orthopedics. C.L.
1984; 253.
6) Kelley, et al. Textbook of Rheumatology. Saunders 1985;
2:1594.
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J. Anthony Parker, MD PhD, Tony_Parker@bidmc.harvard.edu