Paget’s Disease

What is Paget’s disease?

Paget’s disease of bone, also known as osteitis deformans, is a metabolic bone disorder with abnormal bone growth of the aging skeleton. This is characterized by an acceleration in the normal rate of bone turnover—which is our body’s normal act of rebuilding the skeleton throughout life. The abnormal bone growth can occur at a single site or in multiple sites of the skeleton, affecting bone strength and size. It most commonly occurs in just one or a few bones, and because the affected bones have a higher rate of turnover, they are often weaker than unaffected bones. Paget’s disease can affect any bone in the skeleton, but it is most commonly seen in the skull, pelvis, spine and long bones (bones that are longer than they are wide) of the limbs. Paget’s disease commonly presents without symptoms and is frequently found incidentally on X-ray or routine blood work that reveals an elevated serum alkaline phosphatase level, which is a marker of bone turnover. Patients with Paget’s disease of bone who do experience symptoms commonly complain of bone or joint pain. The pain can be directly related to the overgrowth of weak bone or due to related complications that include fractures, deformity, arthritis or neuropathic symptoms from compression of nerves. If the disease affects the legs, as the bones weaken, they can bend, and this can result in bowlegs.

What causes Paget’s disease?

Bones are living tissues that are constantly undergoing a process of remodeling. This process clears out and removes older bone with cells called osteoclasts and replaces it with new bone by cells called osteoblasts. This process relies on balance between osteoclast and osteoblast activity to continue without complications. In Paget’s disease of bone, there is a larger number of the bone removing osteoclasts compared to normal, resulting in a change of the remodeling process that presents with excessive bone that lacks the strength and shape of normal bone. The cause for this imbalance is not exactly known, however it is believed that genetics may predispose a person to developing the condition.

How common is Paget’s disease?

Paget’s disease of bone is a fairly common condition that affects the aging population. Onset of the disease is typically after age 55, with some studies showing a slight predominance in males. Affected populations mostly include those with family members affected by Paget’s disease and individuals with European ancestry. Since Paget’s disease is often asymptomatic, the exact estimate of its occurrence is unknown. Some studies estimate that Paget’s disease occurs in as high as nine percent of older adults within affected populations.

How is Paget’s disease diagnosed?

Most people with Paget’s disease of bone are diagnosed with radiographic imaging. Paget’s disease is commonly diagnosed with X-rays. Examination of X-rays taken of affected bone will show changes that include thickening of the bone with enlargement and deformity. The information gathered from an X-ray of affected bone is enough to make a direct diagnosis of Paget’s disease.

Bone scans may also be used to identify bones that may have Paget’s disease. A bone scan uses a medication that is injected throughout the body through a vein prior to the scan. Areas of the skeleton that are affected by increased bone metabolism are then detected and can be more closely examined with X-rays.

What are the treatment options for Paget’s disease?

Many patients with Paget’s disease of bone do not necessarily require treatment if they do not have symptoms, but treatment is often recommended to reduce the risk of potential complications. When treatment for Paget’s disease is indicated, the first line treatment is pharmacologic using a class of medications called bisphosphonates, often used to treat osteoporosis. These medications work by stopping abnormal bone turnover by inhibiting the osteoclast. Examples of bisphosphonates include: ibandronate (Boniva), zoledronic acid (Zometa, Reclast), alendronate (Fosamax), risedronate (Actonel) and pamidronate (Aredia).

Surgery is another treatment option that is frequently offered to selected patients with Paget’s disease. Orthopedic surgical options that may be considered include joint replacement, long bone deformity correction, fracture fixation, nerve decompression and resection of bony tumors.

Why choose the International Center for Limb Lengthening for treatment of Paget’s disease?

Doctors who treat Paget’s disease