Paget's Disease

What do doctors call this condition?

Osteitis deformans

What is this condition?

Paget's disease is a slow-paced metabolic bone disease characterized by replacement of sound bone with excessive amounts of abnormal bone. The altered bone is fragile and weak, causing painful deformities of both outside shape and internal structure. Paget's disease usually settles in one or several areas of the skeleton (most frequently the lower torso), but occasionally affects many parts of the body.

The disease can be fatal, especially when associated with congestive heart failure (widespread disease creates a continuous need for high cardiac output), bone cancer, or giant cell tumors.

What causes it?

Although the exact cause is unknown, one theory is that an early viral infection (possibly mumps) leaves behind a dormant skeletal infection that erupts many years later as Paget's disease. In the United States, Paget's disease affects approximately 2.5 million people over age 40 (mostly men). In 5% of cases, the involved bone experiences malignant changes .

What are its symptoms?

There may be no symptoms in early stages of the disease. But when pain does develop, it's usually severe and persistent and may limit the person's movement because new bone growth is rubbing on the spinal cord or sensory nerve root. The pain intensifies with exertion.

The list of potential effects includes enlarged forehead, headaches, hunchback, barrel- shaped chest, and bowed legs and other changes in gait. The affected areas are warm and tender, susceptible to breaks, and slow to heal. The disease progresses to add bone growth that interferes with sight, hearing, and balance; complications such as hypertension, gout, and congestive heart failure may develop.

How is it diagnosed?

X-rays, taken even before symptoms appear, can show increased bone expansion and density. A bone scan, which is more sensitive than X-rays, clearly shows early so-called pagetic lesions. Bone biopsy reveals a characteristic mosaic pattern. Blood tests and other laboratory tests aid early diagnosis.

How is it treated?

Primary treatment consists of drug therapy and includes one of the following:

• Cibacalcin, a hormone, given by injection, and Didronel to retard bone resorption (which relieves bone lesions). Although Cibacalcin requires long-term maintenance therapy, there is noticeable improvement after the first few weeks of treatment. Didronel produces improvement after 1 to 3 months.

• Mithracin, an antibiotic that produces remission of symptoms within 2 weeks and biochemical improvement within 1 to 2 months. However, the drug may destroy blood cells or harm kidney function. Self-administration of Cibacalcin and Didronel helps people with Paget's disease lead near-normal lives. Still, they may need surgery to reduce or prevent fractures, correct secondary deformities, and relieve pinched nerves. Aspirin, lndocin, or Advil or Motrin usually controls pain.

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