Paget's Disease of Bone

Photograph used with permission of patient

Clinical features Epidemiology Causes Histology Treatment

Clinical Features

Paget's disease is disorder of bone remodeling which is focal (located in discrete areas in the bones and not throughout the entire skeleton). The abnormal remodeling results in overgrowth or deformity of bone. Paget's disease usually occurs in adults who are older than 55 years old, and there are slightly more men than women with the disease. The disease does not spread, but remains in the same bones as patients grow older. It it most commonly found in the skull, spine, pelvis, femur, and tibia. Whereas some patients have only one bone with Paget's disease, others have Paget's in many bones. The reason for this distribution within the bone remains a mystery.

The image below shows this patient's skeleton as seen on a bone scan. A tiny amout of radioactive medication was injected into her vein; this attaches to areas of bone remodeling. Then a scanner, which is essentially a geiger-counter that can take images, moves over the entire body. Normal bone is light grey and abnormal bone is black. This patient has Paget's disease in the skull, the right shoulder, and the left pelvis and leg. The corresponding xrays show the thick, deformed bones. Compare the normal sides to the abnormal sides, especially at the shoulder and the femur. Click on xrays to see larger view Photographs from Margaret Seton

Paget's disease patients are wonderful people, bright and otherwise healthy, living full lives. Many patients with Paget's disease of bone have no symptoms. This is particularly true if the Paget's disease affects only one bone (monostotic). Other patients have bone pain. In some patients bone pain and crippling deformities of bone occur, requiring treatment with medication and/or surgery.

The joints next to the bones involved with Paget's often develop arthritis. Other complications are the result of bone overgrowth near nerves. For example, if there is Paget's disease in the skull the patient may have problems with hearing. Rare complications include spinal cord paralysis, heart failure, and bone cancer. The blood flow through Pagetic bone is very high, so sometimes the overlying skin is warmer than the skin over non-involved bones.

Since the discovery of new drugs for the treatment of Paget's disease in the last 10 years, patients are being identified and treated earlier. It is hoped that problems from overgrowth of bone will be prevented in this way.

. . . about Paget's disease

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