Paget’s Disease of Bone



Fig. 33.1
Clinical symptoms of Paget’s disease shown by imaging methods. (a) Female patient with more than 20-year history of untreated disease. Significant skull bone changes and complete loss of hearing. (b) Radiography of the patient’s skull with cotton-wool appearance of bone due to lytic lesions. (c) CT scan of the patient’s head with marked thickening of the calvaria



Diagnostic suspicion arises if laboratory and imaging methods reveal elevated serum alkaline phosphatase levels and bone changes. However, in about 10 % of patients, concentration of alkaline phosphatase may be within the reference range. In such case certain markers of bone resorption may be helpful, such as hydroxyproline, pyridinolines, N- or C-telopeptides, P1NP and bone alkaline phosphatase. There is good correlation of bone markers with disease activity, as seen by bone scintigraphy. Calcium and phosphorus levels are commonly within reference ranges, but 20 % of patients may have elevated parathyroid hormone levels, and sometimes elevated uric acid levels.

Due to their typical nature, changes are readily identifiable by imaging techniques. Paget’s disease has several typical features, such as cotton-wool appearance of skull bone, due to enlarged osteolytic region and bone hypertrophy (Fig. 33.1b). Computed tomography and nuclear magnetic resonance imaging are used to assess nerve compression and ill-defined stress fractures and to examine tumour transformation (Fig. 33.1c). Bone scintigraphy is helpful in detecting the scope of the disease (Fig. 33.2). In some patients, scintigraphic changes may appear prior to radiological changes.

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Fig. 33.2
A 56-year-old patient, whole-body bone scintigraphy shows diffusely increased uptake in the left humerus (monostotic form). (a, c) Front view, (b, d) rear view



33.6 Treatment


Major therapy targets are to eliminate pain and restore normal bone metabolism. Treatment of bone remodelling should return lamellar bone formation back to normal and decrease bone vascularity and serum alkaline phosphatase levels. Goals are to slow down the progression of the disease and prevent development of deformities, fractures and osteoarthritis (Table 33.1).


Table 33.1
Main clinical symptoms of the Paget’s disease and their treatment
































Clinical presentation

Symptoms

Treatment

Musculoskeletal

Pain and local increase of temperature

Bone deformitiesa

Osteoarthritisa

Pathological fracturesa

Acetabular protrusiona

Vertebral canal stenosis

Surgical bleeding

Analgesics

Antiphlogistic drugs

Bisphosphonates

Calcitonin

Operative treatment, rehabilitation

Psychological support

Endocrine

Elevated alkaline phosphatase

Normal or hypercalcaemia

Hyperuricaemia

Nephrolithiasis

Bisphosphonates

Vitamin D

Allopurinol

Neurological

Hearing loss and tinnitusa

Dizziness

Damage to cranial nerves

Basilar artery compression

Cranial hypertension

Hearing aids

Bisphosphonates

Calcitonin

Cardiovascular

Retrosternal pain

High-output cardiac failure

Aortic stenosisa

Atherosclerosisa
 

Neoplastic

Sarcoma

Giant cell tumour

Surgical resection


aConditions not indicated for suppressive treatment of Paget´s disease due to its ineffectiveness

Specific treatment of Paget’s disease is indicated in patients with symptoms, in asymptomatic patients with elevated serum alkaline phosphatase levels, preoperatively to reduce bleeding and infrequently in hypercalcaemia, due to the patient’s immobilisation in the polyostotic form of the disease.

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Jul 16, 2017 | Posted by in MUSCULOSKELETAL MEDICINE | Comments Off on Paget’s Disease of Bone
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