Paget’s disease
Also known as osteitis deformans, Paget’s disease is a slowly progressive metabolic bone disease characterized by an initial phase of excessive bone resorption (osteoclastic phase), followed by a reactive phase of excessive abnormal bone formation (osteoblastic phase). The new bone structure, which is chaotic, fragile, and weak, causes painful deformities of both external contour and internal structure.
Paget’s disease usually localizes in one or several areas of the skeleton (most commonly the lower torso); however, occasionally, skeletal deformity is widely distributed. It can be fatal, particularly when it’s associated with heart failure (widespread disease creates a continuous need for high cardiac output), bone sarcoma, or giant cell tumors.
Causes
Paget’s disease occurs worldwide but is more common in Europe, Australia, and New Zealand, where it’s seen in up to 5% of the elderly population.
Although its exact cause is unknown, one theory holds that early viral infection (possibly with mumps virus) causes a dormant skeletal infection that erupts many years later as Paget’s disease. In 5% of the patients, the involved bone will undergo malignant changes.
Signs and symptoms
Clinical features of Paget’s disease vary.
Pain
A patient in the early stages of the disease may be asymptomatic; however, when pain develops, it’s usually severe and persistent and may coexist with impaired movement resulting from impingement of abnormal bone on the spinal cord or sensory nerve root. Such pain intensifies with weight bearing.
Other features
The patient with skull involvement shows characteristic cranial enlargement over frontal and occipital areas (hat size may increase) and may complain of headaches. Other deformities include kyphosis (spinal curvature due to compression fractures of vertebrae), accompanied by a barrel-shaped chest and asymmetrical bowing of the tibia and femur, which can reduce height. Pagetic sites are warm and tender and are susceptible to pathologic fractures after minor trauma. Pagetic fractures heal slowly, and for many patients they never completely heal.
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