Chronic localized bone remodeling disorder characterized by increased bone resorption, bone formation, and remodeling, which may lead to major long bone and skull deformities.
Majority of patients are asymptomatic. Pain is localized to the bone or joint in one third of symptomatic patients, with progressive deformity such as bone bowing and eventual pathological fractures.
Neurological symptoms, due to remodeling of skull, include: hearing loss, chronic facial pain, hydrocephalus and spinal stenosis. Systemic symptoms include high output cardiac failure caused by high blood flow to metabolically active bone sites.
Diagnosis is incidental in the majority of cases. Radiographs have classical appearance. Serum alkaline phosphatase is elevated. Bone biopsy is the only confirmatory diagnostic test, but is rarely indicated.
Symptomatic nondrug therapy includes physical therapy, orthoses, walking aids, hearing aids, and corrective lenses. Nonsteroidal anti-inflammatory drugs and other analgesics are indicated for pain and inflammatory symptoms. Bisphosphonates or calcitonin may be administered to retard excessive osteoclastic activity.
X-ray of Paget disease of proximal femur
From the collection of Camilo Restrepo, Rothman Institute, Philadelphia, PA