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Paget's Disease: Jenn Bray, Lauren Campbell, and Jordan Hamilton

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Paget’s Disease:

Paget’s Disease Jenn Bray Lauren Campbell Jordan Hamilton 6/14/13

General Description¹:

General Description¹ A chronic metabolic condition that causes abnormal bone growth due to overactivity of osteoclastic activity followed by compensatory osteoblastic activity Bone tissue is broken down and absorbed faster than normal, and newly formed bone lacks structural stability This puts the bones at a mechanical disadvantage, with the bones becoming more fragile and brittle, leaving them more susceptible to fractures

Etiology:

Etiology The cause of Paget’s disease is largely unknown Genetic factors, especially gene mutations, are thought to be linked to the cause² Environmental factors, such as viruses³, often contribute to the pathogenesis Possible triggers are calcium deficiency and repetitive loading of bone²

Epidemiology:

Epidemiology Paget’s disease targets five areas of the body: the pelvis (70%), femur(55%), lumbar spine(53%), skull(42%), and tibia (32%) ⁴ The prevalence in the US is 2.3% of the population between the ages of 65 and 74⁵. The highest prevalence of the disease is in Europe, specifically England, France, and Germany⁶. Male to female ratio of incidence is 2:1⁷

Clinical Presentation:

Clinical Presentation Approximately 70-90% of individuals affected with Paget’s do not display symptoms⁷ 40% of patients that do seek medical attention complain of bone pain⁴. Most patients report this pain coming on at night or at rest, or with the use of the affected limb² Bone deformity and warmth of the skin over the affected bone are clinical signs of this disease² Because the disease is normally asymptomatic clinically, the disease is usually detected via radiograph⁴

Differential Diagnosis:

Differential Diagnosis Paget’s disease can often present as osteoarthritis, osteomalacia ⁷ , or degenerative disc disease⁴ Differentiation is performed by radiograph, where Paget’s will present with distinct horizontal radiolucency in the curved convex surface of the bone⁷

Prognosis:

Prognosis If Paget’s disease is detected and treatment initiated in the early stages, prognosis is good It is critical to catch the disease before significant changes occur in the bone Prognosis worsens with the presence of osteogenic sarcomas, which are a complication of the disease²

Treatment:

Treatment The first step to treating Paget’s is management of the disease Pharmocologically Biophosphates : the first drug of choice for treatment. These drugs are used for the treatment of osteoporosis, by reducing bone remodeling and resorption ⁸. Calcitonin: administered when biophosphates are not tolerated. This is a naturally occurring hormone that is involved in calcium regulation and bone metabolism⁹ NSAIDS and Analgesics: to treat the pain Surgery: used if pharmacological agents are not effective There are five main indicators for surgical treatment: Fracture, deformity causing pain, Pagetic arthropathy , entrapment neuropathies and myelopathies, and cancer.

Impact on Function:

Impact on Function Bone pain without an explanation many times leads to depression in these patients⁸ Arthritis and pain resulting from the disease typically limit activities of daily living, as well as physical activities. Patients often suffer from joint pain, decreased range of motion, muscle weakness, and a decrease in cardiac capacity Hip pain that can result from the disease often makes it difficult for the patient to ambulate, leading to decreased speed of gait and sometimes waddling gait¹⁰ Depression and decreased activity often lead to weight gain Due to the decrease in function, Paget’s disease leads to an increase in risk of falls and fractures¹⁰

Role of Imaging:

Role of Imaging Imaging is crucial in diagnosing, evaluating, and monitoring the disease in terms of complication and effect of treatment Standard X-ray, bone scan, CT, and MRI are the major imaging modalities used for patients with Paget’s disease¹¹ Plain radiographs have a high specificity for depicting enlargement of bone, such as sclerosis and bone lesions¹¹

Management:

Management Once diagnosis and impairments are detected, management of Paget’s can progress accordingly Patient education is key in understanding and ensuring proper care to prevent progression of the disease. Physical therapy can be utilized to address the musculoskeletal impairments associated with pain, range of motion and strength deficits.

Best Imaging for Paget’s:

Best Imaging for Paget’s Standard radiograph is the best imaging tool for Paget’s disease due to the ability to show enlargement of the bone, increased radiolucency, and structural deformity.

Alignment:

Alignment smaller in comparison to the left, indicative of malalignment of the pelvis. Shenton’s line differs between the two sides of the pelvis. The iliofemoral line also varies from side to side. T he inclination angle on the right side is greater than the left side, demonstrating greater abduction of the right femur Looking at the sacrum, we can see that it is in left rotation and sidebending Lastly, it appears that there is additional bone laydown outside of the normal structure of the left ilium in the inferior lateral direction In this radiograph, we can see that the right innominate is in anterior torsion. This is based upon the increased radiodensity of the borders of the superior pubic ramus, indicating that it is more anterior¹². The right obturator foramen is http://www.endotext.org/parathyroid/parathyroid15/parathyroidframe15.htm

Bone Density:

Bone Density result of increased osteoblastic activity, a cardinal sign of Paget’s. This sign is also seen in the left acetabulum. The left ilium has a porous appearance, evident by the radiotranslucency of the bone, suggestive of osteoporosis. The most obvious changes in bone density can be seen in the right femoral head and the left innominate. There is increased radiodensity in the right femoral head, which is a

Cartilage Space:

Cartilage Space There is a significant decrease in the joint space of the left hip in comparison with the right. There is almost no space present between the femoral head and the acetabulum. The left sacroiliac joint displays an increase in joint space compared to the right.

Soft Tissue:

Soft Tissue The soft tissue in this radiograph is unremarkable

Biomechanical Impairments:

Biomechanical Impairments Range of motion in this patient will most likely be limited due to the decreased joint spaces Due to the decrease in range of motion, various muscles will be put at a mechanical disadvantage, and therefore they will present as weak As a result of the malalignment of the right pelvis and femur, a possible leg length discrepancy could exist. Abnormal gait may be a problem with this patient, due to the osteoarthritis of the left hip

Physical Limitations:

Physical Limitations This patient will most likely demonstrate weight-bearing pain due to decreased joint space, which in turn will lead to decreased function With ambulation, the impairments seen in this radiograph will lead to decreased speed of gait and decreased endurance Overall, activities of daily living will be challenging for this patient

Bone Scan:

Bone Scan In this bone scan, we can see that the patient is affected in the spine, left pelvis, right hand, left knee, and right ankle Bone scans are useful with Paget’s disease in diagnosis, because the entire body can be seen with one scan, therefore identifying which bones are affected It is also useful in determining if the disease is active or inactive. Additionally, it may identify small fractures in the bone that may go undetected on a radiograph http://www.endotext.org/parathyroid/parathyroid15/parathyroidframe15.htm

Imaging Related Questions:

Imaging Related Questions If a patient presented to your clinic with Paget’s disease diagnosed via radiograph, would you treat the patient or request further imaging studies? If so, what other imaging modalities would you request and why? Your patient was diagnosed with Paget’s disease one year ago via standard radiograph, and presents to physical therapy with the diagnostic radiograph. Would you treat the patient based on these radiological findings, or would you request more recent imaging and why?

References:

References Goodman CC, Snyder TEK. Differential Diagnosis for Physical Therapist: Screening for Referral. 5 th Edition. St. Louis, MO: Elsevier Saunders; 2013. Ralston SH, Langston AL, Reid IR. Pathogenesis and management of Paget’s disease of bone. Lancet . 2008; 372(9633): 155-63 Rebel A, Basle M, Pouplard A, Malkani K, Filmon R, Lepatezour A. Towards a viral etiology for Paget's disease of bone. Metab Bone Dis Relat Res . 1981;3(4-5):235-8. Ralston S. Paget's Disease of Bone. The New England Journal of Medicine. 2013; 368: 644-650 . Altman RD, Bloch DA, Hochberg MC, Murphy WA. Prevalence of pelvic Paget's disease of bone in the United States. J Bone Miner Res . Mar 2000;15(3):461-5 . Poór G, Donáth J, Fornet B, Cooper C. Epidemiology of Paget's disease in Europe: the prevalence is decreasing. J Bone Miner Res . Oct 2006;21(10):1545-9. “Medscape Reference Drugs, Diseases, & Procedures: Paget Disease Clinical Presentation”. Copyright 1994-2013, WebMD LLC. Retrieved on June 14 from http://emedicine.medscape.com/article/334607-clinical .

References:

References Oliveira L, Eslava A. Treatment of Paget's disease of bone. Reumatol Clin . 2012; 8(4): 220-224. “Paget’s Disease of Bone: Treatment and Drugs.” Copyright 1998-2013. Mayo Clinic. Retrieved on June 15 th from http://www.mayoclinic.com / health/ pagets -disease-of-bone/DS00485/DSECTION=treatments-and-drugs Goodman CC and Fuller KS. Pathology: Implications for the Physical Therapist . 3 rd ed. St. Louis, MO: Saunders Elsevier; 2009 . Daphne TJ, Stravroula TJ and Yousuke K. “Imaging of Paget Disease of Bone and Its Musculoskeletal Complications: Review”. American Journal of Roentgenology . 2011; 196: S64-S75 . Agustsson H. Imaging for the physical and occupational therapists. University of St. Augustine. 2013.