Paget disease


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Pa·get dis·ease

(paj'ĕt), [MIM*602080, MIM*167300]
1. a generalized skeletal disease, frequently familial, of older people in which bone resorption and formation are both increased, leading to thickening and softening of bones (for example, the skull), and bending of weight-bearing bones; Synonym(s): osteitis deformans
2. a disease of elderly women, characterized by an infiltrated, somewhat eczematous lesion surrounding and involving the nipple and areola, and associated with subjacent intraductal cancer of the breast and infiltration of the lower epidermis by malignant cells;

Pa·get dis·ease

(paj'ĕt di-zēz')
1. A generalized skeletal disease, frequently familial, of older people in which bone resorption and formation are both increased, leading to thickening and softening of bones (e.g., the skull), and bending of weight-bearing bones.
Synonym(s): osteitis deformans.
2. A disease of old women, characterized by an infiltrated, somewhat eczematous lesion surrounding and involving the nipple and areola, and associated with subjacent intraductal cancer of the breast and infiltration of the lower epidermis by malignant cells.

Paget Disease

DRG Category:553
Mean LOS:5.6 days
Description:MEDICAL: Bone Disease and Arthropathies With Major CC

Paget disease, or osteitis deformans, is a slowly progressing condition of bone structure characterized by increased and disorganized bone turnover. It affects approximately 4% of the U.S. population older than 40. The bones affected vary, but those most commonly involved are the femur, tibia, lower spine, pelvis, and skull. Initially, there is an increase in the number of osteoclasts, which leads to excessive bone resorption and a compensatory increase in osteoblastic activity to repair bone matrix. As a result, the bone is enlarged and distorted, with areas of poor mineralization that resemble a mosaic pattern. Consequently, the bone cannot adequately withstand stresses and strains and is weaker.

Paget disease causes bones to fracture easily, often after only a minor trauma; these fractures heal slowly and often incompletely. If the spine is involved, the vertebrae may collapse, causing paraplegia. If the skull is involved, bony impingements on the cranial nerves can lead to blindness, hearing loss, tinnitus, or vertigo. Other complications of Paget disease include osteoarthritis, hypocalcemia, renal calculi, bone sarcoma, hypertension, and gout. The disease is most life-threatening when it is combined with congestive heart failure because of the need for increased cardiac output to supply increased blood flow to the bones.

Causes

The cause of Paget disease is not known. One theory suggests that it is the result of a slow viral infection, possibly mumps, with a long dormant period. A familial tendency has also been noted. Regional differences suggest that it may have an environmental cause.

Genetic considerations

Paget disease appears to have a hereditary component. Family studies have demonstrated that there are multiple predisposition genes, including TNFRSF11A, TNFRSF11B, and SQSTM1, with additional loci at 5q31, 6p21.3, and 18q23. However, families with affected members have been small, making genetic studies difficult.

Gender, ethnic/racial, and life span considerations

Paget disease occurs most frequently in people over age 40 and is more common in men than in women. Prevalence is difficult to determine because many people have no symptoms. Experts estimate that as many as 10% of people over age 80 have the disease.

Global health considerations

For unknown reasons, Paget disease occurs more often in the northern United States than in southern regions. It is also more common in the North America, Western Europe (except for Scandinavia), and Australia than other regions of the globe. Paget disease is more common in African Americans than in Africans, but whether that difference occurs because of genetic or environmental reasons is unknown.

Assessment

History

Paget disease is frequently discovered as a result of x-rays taken during a routine physical examination. Ask if the patient has experienced deep bone pain. Establish a history of bone fractures. Ask if the patient’s hat size has changed or if he or she has experienced headaches. Elicit any history of decreased hearing or vision or of vertigo. Find out if the patient has noticed any changes in gait while walking or has experienced any difficulty breathing (possibly caused by kyphosis of the spine).

Physical examination

Although most people with Paget disease are asymptomatic, some present with bone pain. Assess the patient’s skeleton for deformities. Inspect the skull for enlargement, particularly in the frontal and occipital areas. Check for cranial nerve compression by testing for decreased hearing and vision, difficulty swallowing, and problems with balance. Examine legs and arms for bowing and subsequent deformities. Ask the patient to walk across the room and back to observe a characteristic waddling gait.

Observe for signs of kyphosis. Note if the patient is bent forward with the chin resting on the chest or has a barrel chest. Kyphosis can compromise chest excursion and cause dyspnea. Palpate the affected areas for increased warmth. Paget disease causes an increase in vascularity of the affected bones, causing the skin temperature to rise. Elderly patients or those with large, highly vascular lesions may develop congestive heart failure because of the heart’s attempt to pump more blood through bones with increased vascularity. Assess the patient’s apical pulse, respirations, and blood pressure. Note the presence of peripheral edema and neck vein distention from right-sided heart failure and pulmonary congestion.

Psychosocial

Because the diagnosis of Paget disease is unexpected, the patient and family may experience increased anxiety levels. There may be fear related to possible falls with fractures and the possibility of self-care deficits. Social isolation may also occur as a result of increasing bone pain and deformities.

Diagnostic highlights

TestNormal ResultAbnormality With ConditionExplanation
X-raysNormal structure of the skeletal systemDisordered and architecturally unsoundDisease causes increased osteoclastic bone resorption
Computed tomographyNormal structure of the skeletal systemEnlarged bones with trabecular (small interconnecting rods of bone making up mass of spongy bone) coarsening and increased cortical thicknessDisease causes increased osteoclastic bone resorption; often used to diagnose complications
Bone scansNormal structure of the skeletal systemIdentifies involved areas of the skeletonDisease causes increased osteoclastic bone resorption

Other Tests: Magnetic resonance imaging, bone biopsy, serum calcium, complete blood cell count, serum alkaline phosphatase, urinary N-telopeptide, α-C telopeptide

Primary nursing diagnosis

Diagnosis

Pain (acute) related to fractures and nerve compression

Outcomes

Comfort level; Pain control behavior; Pain level; Symptom control behavior; Symptom severity; Well-being

Interventions

Pain management; Analgesic administration; Cutaneous stimulation; Heat/cold application; Touch; Exercise therapy; Progressive muscle relaxation

Planning and implementation

Collaborative

medical.
Generally, patients with Paget disease who are asymptomatic require no specific treatment but need careful ongoing monitoring. Calcitonin and bisphosphonates are used to treat Paget disease. Mild pain is usually controlled successfully with NSAIDs. Nonpharmacologic treatment modalities are also prescribed for patients with Paget disease. Heat therapy and massage can help decrease pain. A physical therapy consultation can provide a protocol of simple strengthening and weight-bearing exercises. Braces and other ambulation aids may help support deformities and improve function.

surgical.
Surgery may be needed to reduce or prevent pathological fractures, to correct secondary deformities, and to relieve neurological impairment. Unfortunately, joint replacement is difficult because methyl methacrylate (a gluelike bonding material) does not set properly on bone that is affected by Paget disease.

Pharmacologic highlights

Medication or Drug ClassDosageDescriptionRationale
Alendronate (Fosamax)40 mg/day for 6 mo; may also use etidronate, risedronate, pamidronate, tiludronateBisphosphonates; calcium regulatorSlows rate of bone turnover in pagetic lesions; lowers serum alkaline phosphatase; reduces elevated cardiac output by decreasing vascularity of bone, thereby averting high-output cardiac failure
Calcitonin100 IU SC or IM qd initially and then 50 IU SC three times a weekCalcium regulatorDecreases the number and availability of osteoclasts, thereby retarding bone resorption; relieves bone pain; helps the remodeling of pagetoid bone into lamellar bone

Independent

Focus on reducing pain and immobility, preventing injury, and educating the patient about the disease and treatment regimen. Correlate the patient’s pain with her or his activities and modify schedules as needed. Instruct the patient in the use of relaxation techniques, such as guided imagery and music therapy. Evaluate the patient’s level of functioning and encourage the patient to remain as active as possible. Perform range-of-motion exercises to joints, unless contraindicated, progressing from passive to active exercise as tolerated. Encourage the patient to perform self-care activities independently.

For patients on extended bedrest, reposition the patient frequently and use a flotation mattress. Prevent pressure ulcers by providing meticulous skin care.

Institute measures to prevent injury. Instruct the patient to move slowly and avoid sudden movements. Keep the environment free of clutter. Encourage the use of ambulation aids, such as walkers or canes, as needed. Carefully plan exercise protocols and activity regimens to minimize fatigue.

Evidence-Based Practice and Health Policy

Alonso, N., Albagha, O.M.E., Rios-Visconti, M., & Ralston, S.H. (2012). Identification of novel genetic markers that predict disease severity and complications in Paget disease of bone. Bone, 50, S52–S52. doi 10.1016/j.bone.2012.02.141

  • A study among 771 patients with Paget disease of the bone found that disease severity score was significantly increased among patients with > 16 risk alleles compared to patients with ≤ 16 risk alleles (mean severity scores, 6.24 versus 5.57; p < 0.0001). In this sample, 33.6% carried > 16 risk alleles.
  • Patients with > 16 risk alleles were also more likely than patients with ≤ 16 risk alleles to have a greater number of bones affected (p < 0.009), have received a greater number of treatments for Paget disease (p < 0.001), and have experienced deafness due to skull involvement (10% of patients with > 16 risk alleles versus 5.8% of patients with ≤ 16 risk alleles; p = 0.037).

Documentation guidelines

  • Physical findings: Kyphosis, mobility, gait, presence of skeletal deformities
  • Description of physical pain, comfort measures used, and response to interventions
  • Sensory disturbances: Vision loss, hearing loss
  • Level of tolerance of activity
  • Laboratory results: Serum calcium and alkaline phosphatase levels
  • Reaction to changes in body image

Discharge and home healthcare guidelines

Encourage the patient to follow a recommended moderate exercise program. Suggest the use of a firm mattress or bed board to minimize spinal deformities. Emphasize the importance of attending physical therapy sessions and routine follow-up visits with the physician. Stress the importance of assessing the home environment for safety. Teach the patient to maintain adequate lighting, remove scatter rugs, and keep the home uncluttered. Instruct the patient to avoid abrupt movements and to report any increases in bone pain. Teach the patient to use assistive devices for ambulation.

Be sure the patient understands the dosage, route, action, and side effects of all prescribed medications. Instruct the patient in proper self-injection techniques for calcitonin. Educate the patient about side effects, such as nausea, vomiting, itchy hands, fever, inflammation of the injection site, and facial flushing, as well as the signs and symptoms of hypercalcemia. Teach the patient to take ethane hydroxy diphosphate medication with fruit juice on an empty stomach at least 2 hours before meals. Tell the patient to call the physician if he or she experiences stomach cramps, diarrhea, or new bone pain. Educate the patient who is taking plicamycin about the signs and symptoms of infection. Tell the patient to report any easy bruising, bleeding, nausea, or anorexia. Provide information about the Paget Foundation (165 Cadman Plaza East, Brooklyn, NY 11201).

Paget,

Sir James, English surgeon, 1814-1899.
extramammary Paget disease - Synonym(s): Paget disease
Paget abscess syndrome - an abscess recurrence at the same site after apparent cure.
Paget associated osteogenic sarcoma
Paget cells - relatively large neoplastic epithelial cells.
Paget disease - an intraepidermal form of mucinous adenocarcinoma, most commonly in the anogenital region. Synonym(s): extramammary Paget disease
Paget disease of bone - osteitis deformans. Synonym(s): Paget II syndrome
Paget disease of the nipple - ductal carcinoma.
Paget disease of the penis - carcinoma that develops after balanitis.
Paget juvenile syndrome - Synonym(s): familial osteoectasia
Paget quiet necrosis - necrosis in the superficial layers of the shaft of a long bone.
Paget I syndrome - relationship to or possible extension of mammary duct carcinoma.
Paget II syndrome - Synonym(s): Paget disease of bone
Paget test - to determine whether a mass is a solid tumor or a cyst.
Paget-von Schrötter syndrome - stress thrombosis or spontaneous thrombosis of the subclavian or axillary vein. Synonym(s): effort-induced thrombosis

Pa·get dis·ease

(paj'ĕt di-zēz') [MIM*602080, 167300]
1. A generalized skeletal disease, frequently familial, of older people in which bone resorption and formation are both increased, leading to thickening and softening of bones (e.g., the skull), and bending of weight-bearing bones.
Synonym(s): osteitis deformans.
2. A disease of elderly women, characterized by an infiltrated, somewhat eczematous lesion surrounding and involving the nipple and areola, and associated with subjacent intraductal cancer of the breast and infiltration of the lower epidermis by malignant cells.
References in periodicals archive ?
Vulvar Toker cells: the long-awaited missing link: a proposal for an origin-based histogenetic classification of extramammary Paget disease.
Paget disease of bone (osteitis deformans) is the second most common bone disease (osteoporosis is the most common).
Paget disease of bone is a localized disorder of bone remodeling, with both active and inactive phases, that results in large and deformed bones in various parts of the skeleton.
Currently the patient is receiving medical therapy for the Paget disease.
7) In order to learn more about this rare type of Paget disease and its clinical significance, we identified 7 cases of isolated dermal invasion from overlying epidermal Paget cells in the absence of other areas of stromal invasion and reviewed the clinical, imaging, and pathologic features and subsequent treatment and outcome.
Paget disease of bone (PD) [1] is a relatively common disorder of bone metabolism, affecting -2-5% of the European population above the age of 50 (1).
Finally, Paget disease represents a form of intraepithelial adenocarcinoma and will be discussed in this section as well.
Interleukin 1 (IL-1), IL-6, and tumor necrosis factor as well as growth factors have been implicated in pathologic responses in the skeleton, particularly in osteoporosis associated with estrogen deficiency, hyperparathyroidism, and Paget disease (11, 22, 23).
Mammary Paget disease (MPD) occurs in 1% to 4% of patients with breast cancers and is characterized by infiltration of the nipple epidermis by adenocarcinoma cells.
Similar to the use of serum total ALP measurements to monitor antiresorptive therapy in patients with Paget disease, we tested whether a new immunoassay for bone ALP could be used to monitor the reduction in bone turnover that results from bisphosphonate therapy in postmenopausal osteoporotic women.