Hypertrophic Osteoarthropathy

Martin Charron, M.D.

J.A. Parker, M.D., Ph.D

December 1, 1987

Case Presentation:

A 47 year-old white male presented with coughing for three months, accompanied by painful swollen knees, legs and ankles.

Findings:

A chest X-ray (arrow) and a CT scan (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 HO. 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 HO. 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 HO 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 HO. 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, jap@nucmed.bih.harvard.edu