Paget's Disease: Difference between revisions

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== Definition/Description  ==
== Introduction ==
[[File:Pagets Disease.jpeg|right|frameless]]
Paget's disease of the [[bone]] is a [[Metabolic and Endocrine Disorders|metabolic]] bone disease caused by increased bone resorption followed by excessive unrestricted bone formation, due to activated osteoclasts. The normal bone marrow is replaced by increased&nbsp;and unorganized&nbsp;[[collagen]] and fibrous tissue, which lacks the structural stability of normal bone. This increased bone mass formation leads to complications eg [[Fracture|fractures]], [[Osteoarthritis|arthritis]], deformities, [[Pain Behaviours|pain]], and&nbsp;to a patient's weakened condition. Paget's disease is the second most common metabolic bone disease to [[osteoporosis]]&nbsp;<ref name="Goodman and Fuller">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. St. Louis, Missouri: Saunders Elsevier;2009.</ref><ref name="Josse">Josse RG, Hanley DA, Kendler D, Marie LG, Adachi JD, Brown J. [https://cimonline.ca/index.php/cim/article/view/2897 Diagnosis and treatment of Paget’s disease of bone.] Clinical and Investigative Medicine. 2007 Oct 1:E210-23.</ref><ref name="Seitz">Seitz S, Priemel M, von Domarus C, Beil FT, Barvencik F, Amling M, Rueger JM. [https://link.springer.com/content/pdf/10.1007/s00068-008-8208-4.pdf The second most common bone disease: a review on Paget’s disease of bone.] European Journal of Trauma and Emergency Surgery. 2008 Dec 1;34(6):549-53.</ref>


Also known as Osteitis Deformans, Paget's disease of the bone is a metabolic bone disease caused by increased bone resorption followed by excessive unrestricted bone formation, due to activated osteoclasts. The normal bone marrow is replaced by increased&nbsp;and unorganized&nbsp;collagen and fibrous tissue, which lacks the structural stability of normal bone. This increased bone mass formation leads to complications such as fractures, arthritis, deformities, pain, and&nbsp;to a patient's weakened condition. Paget's disease is the second most common metabolic bone disease to osteoporosis&nbsp;<ref name="Goodman and Fuller">Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. St. Louis, Missouri: Saunders Elsevier;2009.</ref><ref name="Josse">Josse R, Hanley D, Kendler D, Marie LG, Adachi J, Brown J. "Diagnosis and Treatment of Paget's Disease of the Bone".Clin Invest Med August 2007: E210-E223.</ref><ref name="Seitz">Seitz, Sebastian, Priemel, Matthias. “The Second Most Common Bone Disease: A Review on Paget’s Disease of Bone”. European Journal of Trauma and Emergency Surgery. November 2008.</ref>  
The prognosis for patients who are treated is good, especially if the disease is in its early stages. There is no cure for Paget disease but the disorder can be controlled from progressing.<ref name=":0" />


&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;{{#ev:youtube|TwNzgQ0H_9U}}
== Etiology ==
The exact cause of Paget's disease is unknown, though it is thought to be a slow,&nbsp;[[Viral Infections|viral]]&nbsp;bone infection (the family of paramyxoviruses) due to risk factors that include&nbsp;a gene-environment interaction. <ref name="Goodman and Fuller" /><ref name="Josse" /><ref name="Goodman and Snyder">Goodman C, Snyder T. Differential Diagnosis for Physical Therapists: Screening for Referral. 6th ed. St. Louis, Missouri: Saunders Elsevier, 2007.</ref>  Some research suggests that the osteoclastic abnormalities are due to a cytokine is known as IL-6, found solely in the bone marrow of Paget's disease populations.<ref name=":0" /> The prevalence and causes of Paget's disease has&nbsp;been associated with genetic and geographical factors. A positive family history is reported in as many as 40% of patients with Paget's disease&nbsp;<ref name="Goodman and Fuller" /><ref name="Josse" /><ref name="Goodman and Snyder" /><ref name="Schneider">Schneider D, Hofmann MT,Peterson JA.  Diagnosis and Treatment of Paget’s Disease of Bone. American Family Physician. 2002; 65(10):2069-72</ref>. Paget's disease is mostly seen in an autosomal dominant distribution, and there have been three identifiable&nbsp;chromosomal regions associated with Paget's disease. <ref name="Goodman and Fuller" /> Geographically, populations in European, British, and Australian origin, and a migratory influence also play an important role especially in countries which of the early population migrated from Britain (United States, Australia, New Zealand, Canada).<ref name="Goodman and Fuller" />&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;


== Prevalence  ==
== Epidemiology ==
 
Paget disease is usually seen in individuals older than 50 years. It is common in Caucasians of northern European descent. Paget disease is equally common in males and females. In the US, it affects 1-3 million people with most being asymptomatic. The disorder is slightly more common in white males. The disorder usually presents in the 4-5 decade of life but the diagnosis is often made a decade later<ref name=":0">Bouchette P, Boktor SW. Paget disease. InStatPearls [Internet] 2021 Jul 13. StatPearls Publishing. Available:https://www.ncbi.nlm.nih.gov/books/NBK430805/<nowiki/>(accessed 13.5.2022)</ref>.
*After Osteoporosis, Paget's disease is the most common skeletal disorder.&nbsp;Paget's disease affects&nbsp;approximately 2% to 5% of the population older than 40, and seen in 10% of the population over the age of 70 years old. <ref name="Goodman and Snyder">Goodman C, Snyder T. Differential Diagnosis for Physical Therapists: Screening for Referral. St. Louis, Missouri: Saunders Elsevier, 2007.</ref>
*Paget's disease affects men more than woman by a 3:2 ratio.&nbsp;<ref name="Goodman and Snyder" />
*Evidence has shown that&nbsp;genetic factors of 40% of the individuals with Paget's disease have first-degree relatives with Paget's disease. <ref name="Goodman and Snyder" /><ref name="Schneider">Schneider, Doron, Hofmann, Mary T. “Diagnosis and Treatment of Paget’s Disease of Bone”. American Family Physician. May 2002: 65. No.10.</ref>
*There is a prevalence&nbsp;in British cities of about 2% and 8.3% in Lancaster, England. The United States, Great Britain, Australia, and New Zealand have high prevalences because of&nbsp;increased populations&nbsp;along with&nbsp;northern European ancestry. This&nbsp;condition is rare in Asian, African, Scandinavian, Indian, and Japanese&nbsp;populations.&nbsp;<ref name="Chow">Chow, David. Emedicine. Med Web:Paget Disease. http://emedicine.medscape.com/article/311688-overview. Updated December 18,2008. Accessed April 2, 2010.</ref>
*A&nbsp;recent study shows an estimated prevalence in the United States of 1% to 2% with nearly equal distribution between whites and blacks and between sexes. <ref name="Alexander">Hadjipavlou, Alexander G, Gaitanis, Ioannis N. “Paget’s Disease of Bone and its Management”. The Journal of Bone and Joint Surgery. March 2002: 84B. No.2.</ref>
*Recently, the prevalence of Paget's disease has decreased by 50% of the prevalence in 1983.<ref name="Mayo">Medical Foundation for Medical Education and Research. Mayo Clinic: Paget’s disease of bone.http://www.mayoclinic.com/health/pagets-disease-of-bone/DS00485. Updated August 15, 2008. Accessed March 3, 2010.</ref><br>


== Characteristics/Clinical Presentation  ==
== Characteristics/Clinical Presentation  ==
[[File:Paget's disease of Right Hip Bone.jpg|alt=This file is licensed under the Creative Commons Attribution-Share Alike 4.0 International license.|thumb|Paget's R Hip Bone]]A patient with Paget's disease will often present as asymptomatic. However, the clinical presentation of a symptomatic patient varies greatly, due to the different levels of severity of this condition. The spine and pelvis are commonly affected and among the long bones, the [[femur]] is often involved. Symptomatic patients can present with the following:


A patient with Paget's disease will often present as asymptomatic. However, the clinical presentation of a symptomatic patient varies greatly, due to the different levels of severity of this condition.
* [[Pain Assessment|Pain]] involving the bones and joints
 
* Diffuse joint stiffness
*Bone pain presents as&nbsp;constant, deep, and aching.&nbsp;Bones most commonly affected by Paget's disease include: Pelvis, Lumbar spine, Sacrum, Femur, Tibia, Skull, Shoulders, Thoracic spine, Cervical spine, and the ribs.&nbsp;<ref name="Goodman and Fuller" />
* Abnormally enlarged [[skull]]
*Joint pain including swelling and stiffness&nbsp;may present due to the&nbsp;damage&nbsp;of the&nbsp;cartilage lining the joints near the affected bones. <ref name="Mayo" />
* Musculoskeletal deformities
*The most common symptom experienced with symptomatic Paget's disease is an aching pain worse at night that decreases with physical activity. <ref name="Goodman and Fuller" /><ref name="Josse" />
* Loss of hearing (due to the involvement of the petrous temporal bone)
*Muscular pain may present as referred pain from bony structures involved or as a complication due to the mechanical changes from the joint and bone defects. <ref name="Goodman and Fuller" /><ref name="Josse" />
* [[Migraine Headache|Migraines]]
*Neurological pain due to a compressed nerve root or the spinal cord may cause symptoms such as&nbsp;a sharp pain,&nbsp;numbness, tingling, weakness, hearing loss, and double vision. <ref name="Goodman and Snyder" />
* [[Fracture|Fractures]]
*Patients with Paget's disease usually describe their pain as continuous. Different from osteoarthritis, pagetic bone pain usually increases with rest, on weight bearing, when the limbs are warm, and at night<ref name="Schneider" />
* [[Heart Failure|Heart failure]]
*As many as 70% of&nbsp;people with Paget's disease present asymptomatic and is usually detected through radiographs and laboratory tests which are&nbsp;most times&nbsp;ordered for other indications<ref name="Seton" /><ref name="Roux">Roux, Christian, Dougados, Maxime. “Treatment of Patients with Paget’s Disease of Bone”. Disease Management. November 1999: 58 (5):823-830.</ref>
* [[Cranial Nerves|Cranial nerve]] [[neuropathies]]
 
* [[Headaches and Dizziness|Headaches]]
&nbsp;Other clinical presentations may include <ref name="Goodman and Snyder" />:
* Enlarged skull
 
* Skull and jaw deformity
*Bone deformities such as bowlegs, and increased skull size
*Warmth over the affected bone or joint.
*Facial weakness or numbness.
*Loss of bowel or bladder control which may indicate spinal cord damage.
 
== Associated Co-morbidities  ==
 
*Paget's disease has an insidious onset and the disease progresses slowly. When Paget's disease is present in many bones the overactive osteoclasts have the ability to release enough calcium in the blood stream to cause hypercalcemia, which can&nbsp;cause symptoms such as fatigue, weakness, loss of appetite, abdominal pain, and constipation. <ref name="Josse" /><ref name="Goodman and Fuller" />
*Neurological complications due to nerve entrapment syndromes or nerve root compression as they exit the foramina which is narrowed by the increased bone mass of Paget's disease. <ref name="Josse" />
*Hearing loss due to increased temporal bone mass and 8th spinal nerve compression. <ref name="Josse" />
*Bone deformities such as increased skull size and bowing of limbs. <ref name="Josse" />
*People with Paget's disease are also more susceptible to fractures because Pagetic bone is weaker than normal bone.<ref name="Goodman and Fuller" /><ref name="Josse" />
*Cardiovascular problems may arise when 1/3 to 1/2 of the skeleton is involved, heart failure is possible due to an increased cardiac output (this is the most common cause of death in people with advanced Paget's disease).<ref name="Goodman and Fuller" />
*Metabolic complications of Paget's disease may include renal stones, hyperparathyroidism,&nbsp;and hypercalcemia<ref name="Seton">Seton, Margaret. “Diagnosis, Complications and Treatment of Paget’s disease of bone”. Aging Health.</ref><ref name="Roux" />
*A rare complication is sarcoma which is a bone cancer (also known as osteosarcoma or osteogenic sarcoma). This cancer may develop in Pagetic bone, which occurs in less than 1 percent of people with Paget's disease and usually doesn't develop until many years after the onset.<ref name="Goodman and Snyder" /><br>
 
== Diagnostic Tests/Lab Tests/Lab Values  ==
 
*Radiographic Imaging (primary): Plain X-Ray should be performed in at least one skeletal area (usually in a painful area) to help confirm the diagnosis of Paget's disease. X-rays can also diagnose secondary conditions such as a fracture or arthritis. <ref name="Goodman and Fuller" /><ref name="Josse" />
*Isotopic Bone Scan (Scintigraphy) are more sensitive radiographic images which highlight affected areas of Paget's disease, but&nbsp;the Scintigraphy&nbsp;is only positive if the condition is active. <ref name="Josse" />
*Scintigraphy is recommended for both symptomatic and asymptomatic patient's to determine the severity and involvement of the skeletal structures. <ref name="Josse" />
*Scintigraphy can also be used to monitor a patient's therapeutic&nbsp;response to therapy based on the results of a second diagnostic test. <ref name="Goodman and Fuller" /><ref name="Josse" />
*A Bone Biopsy may also be performed (especially if the X-ray and CT scan&nbsp;are negative)&nbsp;to make a differential diagnosis which would rule out hyperparathyroidism, bone metastasis, multiple myeloma, and fibrous dysplasia. <ref name="Josse" />
*Biochemical Tests: Serum Alkaline Phosphatase (SAP) is an enzyme that is produced by bone cells and are excessively produced in bone containing Paget's disease. <ref name="Goodman and Fuller" /><ref name="Josse" />
*In 85% of patients with Paget's disease, SAP levels are elevated. A solid relationship exists between the severity of the disease measured by the scintigraphy, and the increased levels of SAP in untreated Paget's disease. <ref name="Josse" />
*Many other biochemical tests are available which assess bone matrix resorption such Urinary and Serum Deoxypyridinoline, N-telopeptide, and Alpha and Beta C telopeptides, but none of these tests are as readily available as the SAP diagnostic lab test. <ref name="Josse" />
*Diagnosis of Paget's disease may be suspected based on symptoms, but radiographs are the most specific diagnosis test<ref name="Schneider" />
 
&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp; &nbsp;&nbsp;&nbsp;[[Image:Pelvis Paget's disease.jpg|Image:Pelvis_Paget's_disease.jpg]]&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;[[Image:Bowing tibia in patient with Paget's disease.jpg|Bowing Tibia of Patient with Paget's disease]]&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;
 
&nbsp;&nbsp;Radiograph of&nbsp;Pelvis of Patient with Paget's Disease&nbsp;&nbsp;&nbsp;Radiograph of Bowing Tibia in Patient with Paget's Disease
 
== Causes  ==
 
*The exact cause of Paget's disease is unknown, though it is thought to be a slow,&nbsp;viral&nbsp;bone infection due to risk factors that include&nbsp;a gene-environment interaction. <ref name="Goodman and Fuller" /><ref name="Josse" /><ref name="Goodman and Snyder" />
*The prevalence and causes of Paget's disease has&nbsp;been associated with genetic and geographical factors. A positive family history is reported in as many as 40% of patients with Paget's disease&nbsp;<ref name="Goodman and Fuller" /><ref name="Josse" /><ref name="Goodman and Snyder" /><ref name="Schneider" />
*Paget's disease is mostly seen in an autosomal dominant distribution, and there have been three identifiable&nbsp;chromosomal regions associated with Paget's disease. <ref name="Goodman and Fuller" />
*Geographically, populations in European, British, and Australian origin, and a migratory influence also play an important role especially in countries which of the early population migrated from Britain (United States, Australia, New Zealand, Canada).<ref name="Goodman and Fuller" />
 
&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;{{#ev:youtube|dC2TrqcEJEA}}
 
== Systemic Involvement  ==
 
There are many different systems that may be affected by Paget's disease and many associated conditions<ref name="Goodman and Fuller" />:
 
'''Pain'''


*[[Headache|Headache]]  
The [[Lumbar Spine Fracture|lumbar spine]], [[sacrum]], and skull are involved in most cases. Pain is a common feature and is worse with [[Weight bearing|weight-bearing]].  Incomplete fractures are common in Paget disease and seen in the tibia and femur. Even mild injuries can result in [[Insufficiency Fracture|fractures.]] [[Femoral Neck Fractures|Femur fractures]] often involve the subtrochanteric region.<ref name=":0" />
*Muscular
*Radicular
*Skeletal
*[[Osteoarthritis|Osteoarthritis]]


'''Skeletal'''
== Diagnosis ==


*Kyphoscoliosis
Tests to assist in the diagnosis of Paget disease include:
*Bone Bowing (outward bowed femur or forward bowed femur)
*[[Coxa Vara / Coxa Valga|Coxa Varus (waddling gait) _Coxa_Valga]]
*Acetabular protrusion
*Osteoarthritis
*Fractures
*Bone enlargement
*Vertebral compression


'''Muscular'''
* Bone scan
* Bone x-ray
* Elevated markers of bone breakdown like N-telopeptide


*Myalgia
This disease also may also present with the following findings:
*Stiffness
*Weakness


'''Neurological'''
* Elevated ALP (alkaline phosphatase)
* Normal Serum calcium and Phosphate&nbsp;


*Nerve compression conditions including cranial, spinal, and peripheral nerves
Hyperuricemia is common and is due to a high turnover of bone.
*Hearing loss
*Mental confusion due to deteriorated cognitive function


'''Cardiovascular'''
Secondary hyperparathyroidism occurs in about 10% of patients due to inadequate calcium in the face of increased demand.


*Heart failure
Plain x-rays may reveal arthritis or fractures of gross bony lesions.
*Increased vascularity (causing increased skin temperature around affected area)
*Increased cardiac output


'''Other'''
Bone scans can help document the extent of disease and should be used to follow treatment. In addition, a bone scan can pick up early changes in bone even before the patient develops symptoms.<ref name="Goodman and Fuller" />&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;&nbsp;
== Management ==


*Overall fatigue
Although there’s no cure for Paget’s disease of bone, there are treatments available to help people live well and manage the symptoms.
*Vertigo
*Dizziness
*Tinnitus


== Medical Management (current best evidence)  ==
Medications


Medical Management of Paget's disease depends on the symptoms the patient is experiencing. Pain that arises due to elevated SAP (Serum Alkaline Phosphatase)&nbsp;levels responds well to osteoclast inhibitor medications, while pain that arises from nerve irritation (due to bone deformity) should be treated with NSAIDS or analgesics. Many patients have seen good results from combining analgesics and physical therapy, electrical nerve stimulation, hydrotherapy, and acupuncture. <ref name="Goodman and Fuller" /><ref name="Josse" />
* Bisphosphonates are used to slow the progression of Paget’s disease. They help the body control the bone-building process to stimulate more normal bone growth.
* Pain relievers (analgesics) and non-steroidal anti-inflammatory drugs (NSAIDs) – are used to provide temporary pain relief.
* Calcium and vitamin D – are both important for bone health. You can get calcium through the diet and vitamin D through safe exposure to sunlight.  


&nbsp;'''First Line of Treatment'''
Surgery is only offered as an option to patients diagnosed with Paget disease when there is a progression into osteosarcoma. <ref name=":0" />


Due to abnormal osteoclast activity, inhibition of bone resorption&nbsp;with bisphosphonates (ex. Zoledronic acid, Risedronate, Alendronate, Pamidronate, Etidronate Disodium) is the gold standard and first treatment initiated to decrease osteoclastic activity, improve bone density, and increase the strength of the bone itself. Bisphosphonates many times provide long-lasting remissions and treatment response is monitored through assessing the reduction of biomechanical markers such as the SAP levels.<ref name="Goodman and Fuller" /><ref name="Josse" />&nbsp;Patients should intake 1000-1500 mg of calcium and at least 400 U of vitamin D daily. This recommendation is especially important&nbsp;while utilizing&nbsp;bisphosphonate treatments.&nbsp;The goal of this treatment is to cause a full remission (normal SAP levels)&nbsp;and prevent complications of this condition.
There is no specific diet for patients with Paget disease, however those who are prescribed bisphosphonates should ensure adequate intake of calcium and vitamin D.<ref name="Schneider" />  


Patients who may not tolerate bisphosphonates can&nbsp;use Calcitonin, a natural hormone involved in calcium regulation and bone metabolism. Calcitonin is a drug that&nbsp;is administered by injection.<ref name="Goodman and Snyder" />
== Physical Therapy Management ==
[[File:Walking dog.jpg|thumb|Walking, a safe exercise.]]
Treatment Options Include:


'''Second Line of Treatment'''  
* Encourage Client to Stay active – exercise helps to maintain bone health and joint mobility, as well as strengthen muscles. <ref name="Goodman and Fuller" />Aggressive physical activity is not recommended, as the risk of fracture is high. Certain forms of exercise are not suitable for people with Paget’s disease. eg avoid activities such as jogging, running, jumping, and aggressive forward bending and twisting exercises, if the spine is affected by Paget's disease <ref name="Goodman and Fuller" /><ref name="Schneider" />
* Provide a tailored exercises plan, and also provide techniques and/or devices that can help to improve movement, reduce pain and make everyday activities easier. eg a walking stick to reduce the weight placed through affected bones, braces to correct position, foot orthotics to support and correct abnormal foot position or motion.
* Educate on a healthy well-balanced diet – this can help client reach and maintain a healthy weight and reduce your risk of other health problems. Make sure they include calcium-rich foods.
* Teach new ways to manage pain. eg heat packs can help ease muscle pain, cold packs can help with inflammation, gentle exercise can help relieve muscle tension, transcutaneous electrical nerve stimulation (TENS), and massage . Try different techniques until client finds the things that work best for them<ref name="Chow">Chow, David. Emedicine. Med Web:Paget Disease. http://emedicine.medscape.com/article/311688-overview. Updated December 18,2008. Accessed April 2, 2010.</ref>.
* Encourage client to stay at work, it’s good for health and wellbeing. Discuss ways to help client get back to or stay at work<ref>MSK Pagets Disease Available:https://msk.org.au/pagets-disease/ (accessed 13.5.2022)</ref>
* A physical therapist can also assist a patient with Paget's disease in home modifications to make the patient safer with mobility around the home<ref name="Chow" /><br>


Depending on the severity of pagetic changes&nbsp;and degree of pain, NSAIDS and other anti-inflammatories are used to control the pain.&nbsp;Some patients benefit from a combination of medications such as an analgesic and antidepressant. Modalities such transcutaneous electrical nerve stimulation (TENS), hydrotherapy, acupuncture, and physical therapy may also provide pain relief. Orthopedic assistive devices such as canes and shoe lifts may help some patients.
== References ==
 
'''Third Line of Treatment'''
 
Surgical intervention may be indicated if a patient is resistant to pharmacological or orthopedic interventions.&nbsp;A Joint replacement is indicated if severe degenerative joint breakdown is present. If a nerve compression syndrome is present and the patient is nonresponsive to conservative treatment, surgery is required. Other common surgeries for Paget's disease include occipital craniectomy to relieve basilar and nerve compression, and&nbsp;tibial osteotomy if the varus deformity is severe.
 
== Physical Therapy Management (current best evidence)  ==
 
*Pain caused by increased skeletal abnormalities leads to loss of muscle strength, decreased&nbsp;range of motion, and cardiovascular endurance resulting in functional limitations. Physical Therapy and exercise can assist in maintaining muscle strength, flexibility and joint range of motion, increased endurance, and decrease the chances of deconditioning <ref name="Goodman and Fuller" /><ref name="Chow" />
*Strengthening muscles around the joints affected and its adjacent joints can help minimize skeletal complications of Paget's disease <ref name="Goodman and Fuller" />
*&nbsp;Improvements in cardiovascular function and cardiac output also can result from physical therapy improving functional activities such as walking endurance and longer distances <ref name="Goodman and Fuller" />
*A physical therapist may also provide orthotics and other assistive devices to help in management of extremity deformities.&nbsp;The physical therapist can aid in correcting this problem by providing inserts or making appropriate shoe modifications. Bracing may also&nbsp;be&nbsp;beneficial with&nbsp;an open reduction and internal fixation limb surgeries. Assistive devices for ambulation, such as a cane or walker, can help&nbsp;reduce weight-bearing and pain following these surgeries<ref name="Goodman and Fuller" /><ref name="Chow" />
*A physical therapist can also assist a patient with Paget's disease in home modifications to make the patient safer with mobility around the home<ref name="Chow" />
*Modalities can also be beneficial&nbsp;such as superficial heat, transcutaneous electrical nerve stimulation (TENS), and massage, may be helpful for muscle pain, tenderness, and tightness <ref name="Chow" />
*Physcial therapist should advise precautions, if necessary, for example, avoid activities such as jogging, running, jumping, and aggressive forward bending and twisting exercises, if the spine is affected by Paget's disease <ref name="Goodman and Fuller" /><br>
 
== Differential Diagnosis  ==
 
&nbsp;After obtaining a&nbsp;clinical history&nbsp;and several radiographs and laboratory&nbsp;tests the differential diagnosis can narrowed down to a select few including <ref name="Chow" /><ref name="Goodman and Snyder" />:
 
*[[Osteoarthritis|Osteoarthritis]]
*[[Osteoporosis disease|Osteoporosis]]
*Rheumatoid arthritis
*Psoriatic arthritis
*[[Ankylosing Spondylitis|Ankylosing spondylitis]]
*Diffuse idiopathic skeletal hyperostosis
*Pseudogout
*Peyronie disease
*Bone Metastases
*Fibrous Dysplasia
*Blastic Metastases
*Myelofibrosis
*Renal Osteodystrophy
*Fluorosis
*Mastocytosis
*Tuberous Sclerosis
*Calvarial Hyperostosis
 
&nbsp;Many of these conditions may co-exist with Paget's disease.
 
== Case Reports  ==
 
http://www.ejbjs.org/cgi/reprint/58/7/994
 
http://proquest.umi.com.libproxy.bellarmine.edu/pqdweb?index=7&amp;did=1953783091&amp;SrchMode=2&amp;sid=1&amp;Fmt=6&amp;VInst=PROD&amp;VType=PQD&amp;RQT=309&amp;VName=PQD&amp;TS=1271026385&amp;clientId=1870
 
add links to case studies here (case studies should be added on new pages using the [[Template:Case Study|case study template]])<br>
 
[http://www.ejbjs.org/cgi/reprint/58/7/994]
 
== Resources  ==
 
Foundation for Paget's Disease:&nbsp;http://www.paget.org/
 
Medline Plus:&nbsp;http://www.nlm.nih.gov/medlineplus/pagetsdiseaseofbone.html
 
American Academy of Orthopedic Surgeons:&nbsp;http://orthoinfo.aaos.org/topic.cfm?topic=A00076
 
Online Medical Library:&nbsp;http://www.merck.com/mmpe/sec04/ch037/ch037a.html
 
Arthritis Foundation: http://www.arthritis.org/
 
Medicine Net:&nbsp;http://www.medicinenet.com/pagets_disease/article.htm
 
National Institute of Arthritis Musculoskeletal and Skin Diseases:&nbsp; http://www.niams.nih.gov/Health_Info/Bone/Pagets/pagets_disease_ff.asp
 
== Recent Related Research (from [http://www.ncbi.nlm.nih.gov/pubmed/ Pubmed])  ==
<div class="researchbox"><rss>http://eutils.ncbi.nlm.nih.gov/entrez/eutils/erss.cgi?rss_guid=1ZEzhvEsCLGhItsjmf9n08DQ4ElohtgoEj</rss></div>
== References ==


<references />  
<references />  
 
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Latest revision as of 06:04, 26 March 2023

Original Editors - Kevin Schoenfeld from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Kevin Schoenfeld, Admin, Lucinda hampton, Elaine Lonnemann, Dave Pariser, Priya Gulla, Nikhil Benhur Abburi, Kim Jackson, WikiSysop, Uchechukwu Chukwuemeka, Aminat Abolade, Rachael Lowe, Wendy Walker, Mariam Hashem and Claire Knott  

Introduction[edit | edit source]

Pagets Disease.jpeg

Paget's disease of the bone is a metabolic bone disease caused by increased bone resorption followed by excessive unrestricted bone formation, due to activated osteoclasts. The normal bone marrow is replaced by increased and unorganized collagen and fibrous tissue, which lacks the structural stability of normal bone. This increased bone mass formation leads to complications eg fractures, arthritis, deformities, pain, and to a patient's weakened condition. Paget's disease is the second most common metabolic bone disease to osteoporosis [1][2][3]

The prognosis for patients who are treated is good, especially if the disease is in its early stages. There is no cure for Paget disease but the disorder can be controlled from progressing.[4]

Etiology[edit | edit source]

The exact cause of Paget's disease is unknown, though it is thought to be a slow, viral bone infection (the family of paramyxoviruses) due to risk factors that include a gene-environment interaction. [1][2][5] Some research suggests that the osteoclastic abnormalities are due to a cytokine is known as IL-6, found solely in the bone marrow of Paget's disease populations.[4] The prevalence and causes of Paget's disease has been associated with genetic and geographical factors. A positive family history is reported in as many as 40% of patients with Paget's disease [1][2][5][6]. Paget's disease is mostly seen in an autosomal dominant distribution, and there have been three identifiable chromosomal regions associated with Paget's disease. [1] Geographically, populations in European, British, and Australian origin, and a migratory influence also play an important role especially in countries which of the early population migrated from Britain (United States, Australia, New Zealand, Canada).[1]     

Epidemiology[edit | edit source]

Paget disease is usually seen in individuals older than 50 years. It is common in Caucasians of northern European descent. Paget disease is equally common in males and females. In the US, it affects 1-3 million people with most being asymptomatic. The disorder is slightly more common in white males. The disorder usually presents in the 4-5 decade of life but the diagnosis is often made a decade later[4].

Characteristics/Clinical Presentation[edit | edit source]

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Paget's R Hip Bone

A patient with Paget's disease will often present as asymptomatic. However, the clinical presentation of a symptomatic patient varies greatly, due to the different levels of severity of this condition. The spine and pelvis are commonly affected and among the long bones, the femur is often involved. Symptomatic patients can present with the following:

The lumbar spine, sacrum, and skull are involved in most cases. Pain is a common feature and is worse with weight-bearing. Incomplete fractures are common in Paget disease and seen in the tibia and femur. Even mild injuries can result in fractures. Femur fractures often involve the subtrochanteric region.[4]

Diagnosis[edit | edit source]

Tests to assist in the diagnosis of Paget disease include:

  • Bone scan
  • Bone x-ray
  • Elevated markers of bone breakdown like N-telopeptide

This disease also may also present with the following findings:

  • Elevated ALP (alkaline phosphatase)
  • Normal Serum calcium and Phosphate 

Hyperuricemia is common and is due to a high turnover of bone.

Secondary hyperparathyroidism occurs in about 10% of patients due to inadequate calcium in the face of increased demand.

Plain x-rays may reveal arthritis or fractures of gross bony lesions.

Bone scans can help document the extent of disease and should be used to follow treatment. In addition, a bone scan can pick up early changes in bone even before the patient develops symptoms.[1]                                         

Management[edit | edit source]

Although there’s no cure for Paget’s disease of bone, there are treatments available to help people live well and manage the symptoms.

Medications

  • Bisphosphonates are used to slow the progression of Paget’s disease. They help the body control the bone-building process to stimulate more normal bone growth.
  • Pain relievers (analgesics) and non-steroidal anti-inflammatory drugs (NSAIDs) – are used to provide temporary pain relief.
  • Calcium and vitamin D – are both important for bone health. You can get calcium through the diet and vitamin D through safe exposure to sunlight.

Surgery is only offered as an option to patients diagnosed with Paget disease when there is a progression into osteosarcoma. [4]

There is no specific diet for patients with Paget disease, however those who are prescribed bisphosphonates should ensure adequate intake of calcium and vitamin D.[6]

Physical Therapy Management[edit | edit source]

Walking, a safe exercise.

Treatment Options Include:

  • Encourage Client to Stay active – exercise helps to maintain bone health and joint mobility, as well as strengthen muscles. [1]Aggressive physical activity is not recommended, as the risk of fracture is high. Certain forms of exercise are not suitable for people with Paget’s disease. eg avoid activities such as jogging, running, jumping, and aggressive forward bending and twisting exercises, if the spine is affected by Paget's disease [1][6]
  • Provide a tailored exercises plan, and also provide techniques and/or devices that can help to improve movement, reduce pain and make everyday activities easier. eg a walking stick to reduce the weight placed through affected bones, braces to correct position, foot orthotics to support and correct abnormal foot position or motion.
  • Educate on a healthy well-balanced diet – this can help client reach and maintain a healthy weight and reduce your risk of other health problems. Make sure they include calcium-rich foods.
  • Teach new ways to manage pain. eg heat packs can help ease muscle pain, cold packs can help with inflammation, gentle exercise can help relieve muscle tension, transcutaneous electrical nerve stimulation (TENS), and massage . Try different techniques until client finds the things that work best for them[7].
  • Encourage client to stay at work, it’s good for health and wellbeing. Discuss ways to help client get back to or stay at work[8]
  • A physical therapist can also assist a patient with Paget's disease in home modifications to make the patient safer with mobility around the home[7]

References[edit | edit source]

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 Goodman C, Fuller K. Pathology: Implications for the Physical Therapist. 3rd ed. St. Louis, Missouri: Saunders Elsevier;2009.
  2. ↑ 2.0 2.1 2.2 Josse RG, Hanley DA, Kendler D, Marie LG, Adachi JD, Brown J. Diagnosis and treatment of Paget’s disease of bone. Clinical and Investigative Medicine. 2007 Oct 1:E210-23.
  3. ↑ Seitz S, Priemel M, von Domarus C, Beil FT, Barvencik F, Amling M, Rueger JM. The second most common bone disease: a review on Paget’s disease of bone. European Journal of Trauma and Emergency Surgery. 2008 Dec 1;34(6):549-53.
  4. ↑ 4.0 4.1 4.2 4.3 4.4 Bouchette P, Boktor SW. Paget disease. InStatPearls [Internet] 2021 Jul 13. StatPearls Publishing. Available:https://www.ncbi.nlm.nih.gov/books/NBK430805/(accessed 13.5.2022)
  5. ↑ 5.0 5.1 Goodman C, Snyder T. Differential Diagnosis for Physical Therapists: Screening for Referral. 6th ed. St. Louis, Missouri: Saunders Elsevier, 2007.
  6. ↑ 6.0 6.1 6.2 Schneider D, Hofmann MT,Peterson JA. Diagnosis and Treatment of Paget’s Disease of Bone. American Family Physician. 2002; 65(10):2069-72
  7. ↑ 7.0 7.1 Chow, David. Emedicine. Med Web:Paget Disease. http://emedicine.medscape.com/article/311688-overview. Updated December 18,2008. Accessed April 2, 2010.
  8. ↑ MSK Pagets Disease Available:https://msk.org.au/pagets-disease/ (accessed 13.5.2022)
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