Paget's Disease Flashcards
Definition/Description
Also known as Osteitis Deformans, Paget’s disease of the bone is a metabolic bone disease caused by increased bone resorption followed by excessive unrestricted bone formation, due to activated osteoclasts.
The normal bone marrow is replaced by increased and unorganized collagen and fibrous tissue, which lacks the structural stability of normal bone. This increased bone mass formation leads to complications such as fractures, arthritis, deformities, pain, and to a patient’s weakened condition. Paget’s disease is the second most common metabolic bone disease to osteoporosis
Prevalence
After Osteoporosis, Paget’s disease is the most common skeletal disorder. Paget’s disease affects approximately 2% to 5% of the population older than 40, and seen in 10% of the population over the age of 70 years old. It affects more men than woman by a ratio of 3:2.
The disease also show some genetic involvement, with around 40% of individuals diagnosed with paget’s disease have first degree relatives suffering from the same
Characteristics/Clinical Presentation
A patient with Paget’s disease will often present as asymptomatic. However, the clinical presentation of a symptomatic patient varies greatly, due to the different levels of severity of this condition.
Bone pain presents as constant, deep, and aching. Bones most commonly affected by Paget’s disease include: Pelvis, Lumbar spine, Sacrum, Femur, Tibia, Skull, Shoulders, Thoracic spine, Cervical spine, and the ribs.
Increased focal temperatuture due to hyperaemia
Joint pain including swelling and stiffness may present due to the damage of the cartilage lining the joints near the affected bones.
The most common symptom experienced with symptomatic Paget’s disease is an aching pain worse at night that decreases with physical activity.
Muscular pain may present as referred pain from bony structures involved or as a complication due to the mechanical changes from the joint and bone defects.
Neurological pain due to a compressed nerve root or the spinal cord may cause symptoms such as a sharp pain, numbness, tingling, weakness, hearing loss, and double vision.
Patients with Paget’s disease usually describe their pain as continuous. Different from osteoarthritis, pagetic bone pain usually increases with rest, on weight bearing, when the limbs are warm, and at night
As many as 70% of people with Paget’s disease present asymptomatic and is usually detected through radiographs and laboratory tests which are most times ordered for other indications
Other clinical presentations may include :
Bone deformities such as bowlegs, and increased skull size
Warmth over the affected bone or joint.
Facial weakness or numbness.
Loss of bowel or bladder control which may indicate spinal cord damage
Associated Co-morbidities
Paget’s disease has an insidious onset and the disease progresses slowly. When Paget’s disease is present in many bones the overactive osteoclasts have the ability to release enough calcium in the blood stream to cause hypercalcemia, which can cause symptoms such as fatigue, weakness, loss of appetite, abdominal pain, and constipation.
Neurological complications due to nerve entrapment syndromes or nerve root compression as they exit the foramina which is narrowed by the increased bone mass of Paget’s disease.
Hearing loss due to increased temporal bone mass and 8th spinal nerve compression.
Bone deformities such as increased skull size and bowing of limbs.
People with Paget’s disease are also more susceptible to fractures because Pagetic bone is weaker than normal bone.
Cardiovascular problems may arise when 1/3 to 1/2 of the skeleton is involved, heart failure is possible due to an increased cardiac output (this is the most common cause of death in people with advanced Paget’s disease).
Metabolic complications of Paget’s disease may include renal stones, hyperparathyroidism, and hypercalcemia
A rare complication is sarcoma which is a bone cancer (also known as osteosarcoma or osteogenic sarcoma). This cancer may develop in Pagetic bone, which occurs in less than 1 percent of people with Paget’s disease and usually doesn’t develop until many years after the onset