Osteitis Deformans

Common Name: Paget's Disease

Age of Onset: Paget's Disease is most commonly found in people after they reach 50 years of age, and it is rarely diagnosed those younger than 20 years.

Duration: Paget's Disease is a chronic disease, that has no cure. Although it does not have a cure, Paget's Disease can be treated to minimize the disease's painful effects.

Males/Females/Equal: Paget's Disease is more commonly found in males than in females, the male-to-female ratio about 1.8:1.

Particular Ethnic Group: Paget's disease is more commonly found in the northern European ancestry and British migrant population in countries such as Great Britain, the United States, Australia, and New Zealand. It is particularly common in England. The disease is rare in Asia, Africa, Scandinavia, India, and Japan.

Cause: In Paget's disease, both osteoclasts and osteoblasts become overactive in some areas of bone, and the rate at which bone is broken down and rebuilt in these areas increases. These overactive areas enlarge and become structurally abnormal and weak.

The exact cause of Paget's Disease has not been pin-pointed. Probable causes of Paget's Disease include genetics, the environment, viral infection, inflammation, elevated parathyoid hormone levels, idiopathic hypoparathyroidism, autoimmune, connective tissue, and vascular disorders.

Symptoms: Paget's Disease affects everyone differently, and most often the patient will present zero symptoms. Paget's disease commonly affects the skull, the spine, the pelvis, and the bones of the limbs. The disease may affect a few specific areas of the body or it may be widespread. The symptoms of Paget's Disease will depend on the affected body part.

Some symptoms of Paget's Disease may include:

• Pain in the affected bones (maybe constant, aching, deep, and severe at night)
• Damaged cartilage lining the joints near the affected bones, which may lead to osteoarthritis (OA)
• Severe pain from nerve compression, caused by the enlarged bones compressing the spinal cord. Pressure on a nerve can also cause numbness, tingling, weakness, hearing loss and double vision.
• Warmth in your skin over the affected area
• Neurological problems, such as hearing loss, headache and rarely, vision loss
• Bone deformities, such as bowlegs and enlarged head size
• Fractures

Prognosis (describe the progression of the disease): Paget's Disease causes the osteoclasts to become more active than osteoblasts, and so the bone remodeling and bone growth becomes off balance with more bone absorption than normal. The osteoblasts try to keep up by making new bone, but they overreact and make excess bone. The new-formed bone as a result is abnormally large, weak, brittle, and deformed. The patient's bones and joints then are prone to fractures, bowing, deformities, arthritis, inflammation, and nerve compression.

Treatment Options (Rx): Treatment to Paget's Disease varies greatly. If the patient has no symptoms, then treatment will not be adminstered. However treatment greatly depends on the area that is affected, if a high-risk bone is affected by Paget's (the skull or spine), then the doctor will recommend treatment to prevent OA, heart failure, and sarcoma. Treatment will alleviate some of the pain and halt bone damage due to Paget's, in some cases even cause remission of the disease. The different forms of treatment include:

• Bone-regulating medication: A.) Bisphosphonate (risedronate: Actonel, alendronate: Fosamax, and pamidronate: Aredia, and zoledronic acid: Reclast), used to treat osteoporosis and to increase bone density, or B.) Calcitonin (Miacalcin), the naturally occuring hormone for calcium regulation and bone metabolism.
• Arthritis medication, to reduce pain or inflammation: A.) Nonsteroidal anti-inflammatory drugs (NSAIDs), which reduce the pain and inflammatory effects of arthritis or B.) Acetaminophen (like Tylenol), to reduce the arthritis pain.
• Surgery may be required to help fractures heal, to replace joints damaged by severe arthritis or to realign deformed bones. If Paget's disease affects your spine or your skull, you may need surgery to reduce pressure on nerves and prevent serious complications.
• Daily diet with 1000-1500 mg of calcium and 400 U of vitamin D.
• Exercise to increase skeletal strength and health
• Rehabilitation programs, which include physical therapy, modalities, occupational therapy, and speech therapy

Links:

• <http://www.merck.com/mmhe/sec05/ch061/ch061a.html>
• <http://orthoinfo.aaos.org/topic.cfm?topic=A00076>
• <http://www.mayoclinic.com/health/pagets-disease-of-bone/DS00485>
• <http://www.niams.nih.gov/Health_Info/Bone/Pagets/default.asp>
• <http://emedicine.medscape.com/article/311688-overview>
• <http://www.paget.org/page.asp?page=Patient/paget.asp&title=Paget%27s+Disease+of+Bone&nav_user=Patient>
• <http://myweb.lsbu.ac.uk/dirt/museum/margaret/456-842-1641250.jpg>