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Paget's Disease

Nina J Abramson, MD
Barbara N Weissman, MD

October 21, 1994

Presentation

Patient 1: A 78-year-old woman presented to emergency services with right hip pain.

Patient 2: A 65-year-old man presented with pelvic bone pain and fatigue. (Case courtesy of Dr. Stephen Eustace.)

Imaging Findings

Patient 1
Patient 2

An anteroposterior (AP) radiograph of the pelvis of Patient 1 shows increased bone density of right ilium, ischium and pubic rami. The trabecula are coarse. Both iliopectineal (arrows) and right ilioischiatic lines are thickened.

An AP view of the pelvis of Patient 2 demonstrates marked enlargement of iliopectineal (short arrows, see link below) and ilioischiatic lines; this is greater on the left than on the right. There is an increase in the bone density of the ilium, ischium and L4, cortical thickening, bony expansion, and coarsened trabecular pattern. Also note the lytic destruction of the right sacrum and ilium (long arrow, see link below) with a subtle soft tissue mass projecting into the right side of the pelvis. (Arrows)

Differential Diagnosis

Increased skeletal radiodensity may be seen in a large number of onditions, including Paget's disease, bone metastasis (e.g. prostatic carcinoma), myelofibrosis, renal osteodystrophy, fibrous dysplasia, fluorosis, and tuberous sclerosis, among others. Additional findings characteristic of these disorders, however, assist in their recognition. For example, hepatosplenomegaly is found in myelofibrosis, subperiosteal and subchondral bone resorption and the "rugger-jersey" spine in renal osteodystrophy, bowing deformities and "ground glass" appearance in fibrous dysplasia, and focal radiodensity in tuberous sclerosis. Although bony sclerosis of Paget's disease can mimic osteoblastic metastasis, the asymmetric distribution, thickened trabecula and cortices, and the enlargement of the involved bone are all typical of Paget's disease.

Diagnosis

Paget's disease of pelvis

Discussion

Paget's disease, also known as osteitis deformans , is present in 3% of people over the age of 40 and in 10% over 80 in the US. Men are affected twice as often as women. Worldwide, the disease has higher incidence in Northern latitudes, particularly in Anglo-Saxons, and is rare in Orientals. Although the etiology is not entirely clear, a viral cause has been postulated.

Paget's disease is characterized by excessive and abnormal remodeling of bone leading to a "mosaic" appearance on a pathological examination. Active or osteolytic phase of the disease is associated with aggressive bone resorption and replacement of hematopoietic bone marrow by fibrous connective tissue. This is characterized radiographically as the "blade of grass" leading edge of a lucent lesion in long bones and "osteoporosis circumscripta" in the skull. During the inactive or quiescent phase, bone turnover is decreased.

Paget's disease is usually polyostotic and asymmetric and begins at the ends of long bones in nearly all cases (tibia is a rare exception). Particularly characteristic is the involvement of the pelvis (30 to 75% of cases), sacrum (30 to 60%), spine, especially lumbar (30 to 75%), and skull (25 to 65%). Proximal long bones, particularly the femur, are affected in 25% of patients.

Complications of Paget's disease include stress fractures, sarcomatous transformation into osteosarcomas, chondrosarcomas, and fibrosarcomas, and giant cell tumors (especially of the skull and facial bones). Patients with Paget's disease may also suffer from neurologic deficits resulting from impingement of the spinal cord and foramina. Paget's patients may also develop deformity of the base of the skull (platybasia), high output congestive heart failure due to hyperemia and increased blood flow in Pagetic bone, articular abnormalities secondary to osteoarthritis due to abnormal subchondral bone, and degenerative joint disease.

References

1. Resnick D. Bone and Joint Imaging. Philadelphia: WB Saunders, 1989; 603-614.

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