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Paget Disease of the Bone

Osteitis Deformans

Osteitis deformans, also known as Paget's disease, is a condition frequently observed in elderly patients, despite the fact that it's prevalence has decreased over the last 20 years. With unclear etiology, this pathological entity is characterized by abnormal behavior of osteoclasts and osteoblasts, leading to pathological bone formation. The disease may involve one or more sites and may progress to fractures, osteosarcoma and cardiovascular abnormalities.


Presentation

This disease has an ongoing character, may involve one or more bones at first, but secondary dissemination does not occur.

Patients may be asymptomatic or may have complaints such as bone pain, that is dull, quasi-permanent and accentuated during the night and with weight burden. Uninvolved areas may also hurt, because of the redistribution of the mechanical forces.

Involved bones may present with deformities like bowing. With skull implication, symptoms like headaches, hearing loss, vertigo, tinnitus, changes in vision and cranial nerve palsies occur. If the cranium descends onto the cervical spine, patients experience paresthesias and bowel or bladder incontinence. Other cranial complications include hydrocephalus and subsequent brain compression symptoms: syncope, nausea, ataxia, dementia.

Facial bones may also undergo changes, leading to occlusion abnormalities and loss of teeth.

As axial bones are more frequently affected, patients usually describe lower back, cervical or hip pain. Fractures and arthritis are not uncommon, but if the pain is constantly increasing and the surrounding tissues become swollen, a radiography should be performed in order to exclude osteosarcoma.

Osteitis deformans is characterized by increased vascularization of the affected areas. This can lead to high-output heart failure in patients with decreased contractility reserve [1]. Arteriosclerosis and mitral and aortic valve calcifications have been shown to be related to disease progression and further worsen cardiovascular status [2] [3] [4].

Asymptomatic
  • Article Figures/Media Metrics Paget's disease of bone may present with bone pain but is often asymptomatic.[doi.org]
  • Asymptomatic patients also have no clinical signs of the disease.[symptoma.com]
  • A strong case can also be made for treating asymptomatic patients in whom the location of the disease puts them at risk of future complications.[mja.com.au]
  • Some patients are asymptomatic, whereas others develop complications such as pain, osteoarthritis, fracture, deformity, deafness, and nerve compression syndromes.[ncbi.nlm.nih.gov]
Congestive Heart Failure
  • Symptoms of congestive heart failure (as a large part of the body's blood may be diverted to the diseased bone).[musclesandjoints.com]
  • heart failure due to shunting of blood through warm skin (bone is hypervascular and hot) Leontiasis ossea : cranium too heavy to lift Platybasia : invagination of base of skull due to weak bone, compression of posterior fossa structures Associated neoplasms[pathologyoutlines.com]
  • Also excluded were those with a history of severe congestive heart failure ( n 7) or inadequate medical or pathology records ( n 15).[nature.com]
  • A patient can rarely develop congestive heart failure because of the increased blood flow. Increased calcium levels from the bone remodeling process can cause kidney stones.[emedicinehealth.com]
Limb Pain
  • Limb pain may be due to the lesion itself or result from nerve root compression.[veterans.gc.ca]
  • Continue discussing phantom limb pain with client.[slideplayer.com]
Fatigue
  • 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 cause symptoms such as fatigue, weakness, loss of appetite, abdominal pain, and constipation[physio-pedia.com]
  • ., enlarged head, bowed legs, reduced height) pain or numbness (from pressure on nerves) headache hearing loss (when abnormally formed skull bones press on the nerve) warmth over an affected area fatigue Unless Paget's disease shows up on an X-ray taken[medbroadcast.com]
  • Causes of Fracture: Fatigue- muscles are less supportive to bone, therefore, can’t absorb the force being exerted. Bone neoplasms- cellular proliferations of malignant cells replace normal tissue causing weakened bone.[slideplayer.com]
Tinnitus
  • With skull implication, symptoms like headaches, hearing loss, vertigo, tinnitus, changes in vision and cranial nerve palsies occur. If the cranium descends onto the cervical spine, patients experience paresthesias and bowel or bladder incontinence.[symptoma.com]
  • Changes in the skull may lead to inner ear damage, resulting in deafness, ringing in the ears (tinnitus), or impaired balance (vertigo). It may also result in headaches.[nmihi.com]
  • Deafness and tinnitus may be due to compression of cranial nerve VIII, effects on ear ossicles (eg, stapes fixation) and cochlear dysfunction. Less common Spinal stenosis . Nerve compression syndromes and cauda equina syndrome .[patient.info]
  • Spinal stenosis OA of neighbouring joints Neurological Basilar invagination Deafness, spinal stenosis/vascular steal are all common Cerebellar dysfunction Obstructive hydrocephalus Cranial nerve palsies Spinal stenosis/cauda equine syndrome Deafness Tinnitus[doi.org]
Osteoporosis
  • Abstract Paget's disease of bone is important in geriatric populations because it is the second most common bone disorder after osteoporosis.[ncbi.nlm.nih.gov]
  • A: Lateral radiograph of the skull shows the thickened skull and osteoporosis circumscripta of both lateral temporal bones ( sho... FIG. 122.5.[5minuteconsult.com]
  • Osteoporosis screening: topic overview Health Link B.C. Osteoporosis Canada recommends everyone over age 65 have routine bone density tests. Start routine testing earlier if you are at increased risk for broken bones.[mcmasteroptimalaging.org]
  • Paget's disease of bone is important in geriatric populations because it is the second most common bone disorder after osteoporosis.[jhu.pure.elsevier.com]
  • […] consulting fees on behalf of his institution from Merck and Novartis, lecture fees on behalf of his institution from Amgen and Eli Lilly, and payment on behalf of his institution from Watson Pharmaceuticals for expert testimony relating to ibandronate use in osteoporosis[doi.org]
Bone Pain
  • Bisphosphonate therapy is primarily indicated for patients who have bone pain arising from increased metabolic activity in affected bones.[ncbi.nlm.nih.gov]
  • They are thought to reduce bone turnover, improve bone pain, promote healing of osteolytic lesions and restore normal bone histology [ 1 ] .[patient.info]
Hip Pain
  • Therefore, it is not unexpected that hip pain is one of the major presenting complaints. Analysis of the radiographs of 25 hips with one or more articular sufaces involved by Paget's disease demonstrated narrowing in 24.[ncbi.nlm.nih.gov]
  • Review Topic QID: 107772 3 Paget’s disease (osteitis deformans) M2 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (M2.OR.69) A 67-year-old male presents with left hip pain.[step2.medbullets.com]
  • As axial bones are more frequently affected, patients usually describe lower back, cervical or hip pain.[symptoma.com]
  • Paget's disease of bone in the pelvis can cause hip pain. Skull. An overgrowth of bone in the skull can cause hearing loss or headaches. Spine. If your spine is affected, nerve roots can become compressed.[mayoclinic.org]
Saber Shin
  • ., bowing of legs ( saber shin ) Skull involvement (in 40% of cases) Skull enlargement ( increasing hat size ) Cranial nerve deficits Impaired hearing Headache Cauda equina syndrome, nerve root compression Pathological f ractures : Chalk-stick fractures[amboss.com]
Arthralgia
  • The most common side effects of pamidronate are a transient “flu-like” syndrome of fever, myalgia and arthralgia. Serious side effects are rare, but uveitis and acute renal failure have been reported.[mja.com.au]
Angioid Streaks of the Retina
  • In addition to bowing of legs and cranial enlargement, signs can include a foreshortened spine and angioid streaks in the retina.[etsu.edu]
Frontal Bossing
  • Bone deformity (including sabre tibia (bowing of the tibia), kyphosis, frontal bossing of the skull, an enlarged maxilla, an increase in head size). Pathological fractures (may produce heavy bleeding from the very vascular bone).[patient.info]
  • Skull involvement may produce enlargement of the head characterized by more evident frontal bossing and dilated superficial cranial muscles. If the facial bones are involved as well, a typical leonine appearance may be Noted.[veterans.gc.ca]
  • Paget's disease affecting the skull may cause frontal bossing, increased hat size, and headaches.[en.wikipedia.org]
Headache
  • With skull implication, symptoms like headaches, hearing loss, vertigo, tinnitus, changes in vision and cranial nerve palsies occur. If the cranium descends onto the cervical spine, patients experience paresthesias and bowel or bladder incontinence.[symptoma.com]
  • If they do occur, symptoms may include: Bone pain, joint pain or stiffness, and neck pain (the pain may be severe and present most of the time) Bowing of the legs and other visible deformities Enlarged head and skull deformities Fracture Headache Hearing[nlm.nih.gov]
  • Clinical symptoms: headache, bowing and shortening of the legs, the arms appear relatively long, enlargement of the head (hat and dentures no longer fit), and in extreme cases a leontine facies develops (leontiasis ossea).[med-college.hu]
  • It may also result in headaches. Enlarged vertebrae can press on the spinal cord, causing pain or paralysis of the legs. Pelvis involvement can cause severe arthritis of the hips. Affected bones are prone to fracture.[nmihi.com]
Vertigo
  • Brain compression results in ataxia or dementia, while cranial nerve VII compression leads to deafness and vertigo. Patients may also present fractures as the first sign of disease.[symptoma.com]
  • Changes in the skull may lead to inner ear damage, resulting in deafness, ringing in the ears (tinnitus), or impaired balance (vertigo). It may also result in headaches.[nmihi.com]
  • […] cartilage of affected joints, most commonly: spine cervical osteoarthritis thoracic osteoarthritis lumbar osteoarthritis ankle knee hip Headaches (from skull involvement) Conductive and/or sensorineural hearing loss (from temporal bone involvement) Vertigo[veterans.gc.ca]
  • Other neurological syndromes are uncommon, but include vertigo, spinal cord compression, local compression syndromes, such as cranial nerve palsies, and, rarely, hydrocephalus or brainstem compression from basilar invagination.[mja.com.au]
Dizziness
  • If the disease occurs in bones of the skull, it can cause an enlarged head, hearing loss, headaches, and dizziness.[icdlist.com]
  • The signs and symptoms displayed by Paget's Disease of the Bone could include: Bone pain Deformed bones, causing Bowed legs Enlarged skull Enlarged hips Curved backbone Easy fractures Headache, dizziness Vision and hearing defects Leg pain Numbness Weakness[dovemed.com]
  • Case report A 55-year-old female reported to Department of Oral and Maxillofacial Surgery with the chief complaint of a gradual increase in the size of the upper jaw since 20 years, along with headache and dizziness since 4 years.[amsjournal.com]
  • Enlarged bones in the head can put pressure on nerves leading to tinnitus (ringing or noises in the ear), dizziness, and headaches. Paget's disease usually progresses slowly.[medbroadcast.com]
Waddling Gait
  • On clinical examination, patient was short stature, hunched back with a slow waddling gait. Patient had an enlarged cranium with broad, prominent forehead and severely protrusive, expanded maxilla, caused obliteration of nasolabial sulci.[amsjournal.com]
  • gait) _Coxa_Valga Acetabular protrusion Osteoarthritis Fractures Bone enlargement Vertebral compression Muscular Myalgia Stiffness Weakness Neurological Nerve compression conditions including cranial, spinal, and peripheral nerves Hearing loss Mental[physio-pedia.com]

Workup

Asymptomatic patients also have no clinical signs of the disease. Physical examination may, however, reveal deformed or bowing bones, kyphosis (that may cause spinal cord compression and injury), warm teguments over the affected area, increased skull circumference, facial deformity or narrowing of the airways. Brain compression results in ataxia or dementia, while cranial nerve VII compression leads to deafness and vertigo. Patients may also present fractures as the first sign of disease.

Blood workup should include serum and bone-specific alkaline phosphatase, procollagen I N -terminal peptide and serum total acid phosphatase, all of which may be increased [5]. Even if serum alkaline phosphatase is normal, the disease cannot be excluded. Calcium and phosphate levels are usually normal in mobile patients but may be high if hyperparathyroidism coexists. Secondary hyperparathyroidism may appear due to increased calcium demand. Hyperuricemia has also been described [6].

Urine should be examined for specific markers like hydroxyproline, deoxypyridinoline, C-telopeptide (CTx) and N-telopeptide (NTx) [7]. Alpha-CTx/beta-CTx ratio is high in active disease patients and is a suitable marker to monitor therapy [8].

Plain radiographs are used both for disease and complication diagnose and monitoring, but the gold standard in documenting the extent of the pathological process is represented by Tc99m-MDP scintigraphy, despite the fact that this method is less specific than classical radiology. Computer tomography and magnetic resonance imaging are useful when trying to evaluate malignant transformation, nerve compression, and hydrocephalus.

Radiographs show osteolysis and aberrant bone formation, sclerotic areas, ankylosis, remineralization or heterogeneous ossification [9].

Bone biopsy reveals the typical mosaic pattern [10] that appears as a result of osteolysis, bone formation, and osteosclerosis. The architecture is disorganized and vascularization is abundant. In time, osteosclerosis becomes the most prominent feature.

Nephrolithiasis
  • Nephrolithiasis and pyelonephritis with cortical and perinephric abscesses were also reported.[ncbi.nlm.nih.gov]
  • […] syndrome Para or quadriplegia Cardiovascular Increased cardiac output All uncommon Congestive cardiac failure Aortic stenosis Endocardial calcification Atherosclerosis Metabolic Hypercalcaemia All are rare Hyperuricaemia Immobilization hypercalciuria Nephrolithiasis[doi.org]
Increased Bone Density
  • bone density, increased microfractures, loss of distinction between cortex and medulla; may have sharp demarcation between normal and affected bone; may extend into soft tissue if florid disease Radiology images Contributed by Dr.[pathologyoutlines.com]
  • These medications are intended to increase bone density and reduce the activity of Paget's disease. [1] [2] [4] Zoledronate is the preferred intravenous bisphosphonate, but pamidronate is also a well tolerated intravenously administered drug for treatment[physio-pedia.com]
Alkaline Phosphatase Increased
  • The amount of blood serum alkaline phosphatase increased. A diseased bone increased demand for oxygen and nutrients causes increased load on the heart.[medicalformat.com]

Treatment

  • Treatment Paget’s disease is treatable, but not all patients require treatment (see Box 4 ).[mja.com.au]
  • We suggest treatment with a bisphosphonate before surgery on pagetic bone.[ncbi.nlm.nih.gov]
  • Some start treatment if alkaline phosphatase levels are raised; others only start treatment if there are symptoms. Specific treatment is required for complications. Because of the risk of osteosarcoma, patients should be monitored indefinitely.[patient.info]
  • Treatment The pharmacological treatment of PDB is targeted at reducing osteoclast function ( Table 2 ). Other treatment is aimed at relieving symptoms and complications of the illness.[doi.org]

Prognosis

  • Prognosis of Paget’s Disease (osteitis deformans) With effective treatment prognosis is good and long term remissions can be achieved.[healthengine.com.au]
  • The serum phosphatase is a most useful prognostic guide in a disease with a generally poor prognosis. Magnesium metabolism in relation to bone sarcoma requires further study.[online.boneandjoint.org.uk]
  • Prognosis This depends on the extent and degree of disease activity. Remission may be possible with successful treatment. Those who develop osteosarcoma have a very poor prognosis. History James Paget was born in Great Yarmouth in Norfolk in 1814.[patient.info]
  • Irrespective of the site of involvement, the histopathologic features, or the mode of treatment, the prognosis was extremely poor. The 5-year survival rate was 7.9%, with a mortality rate of 86.8% 36 months after diagnosis.[journals.lww.com]
  • #pic# #pic# Differential diagnosis Fibrous dysplasia, cemento-osseous dysplasias, chronic suppurative osteomyelitis, osteosarcom Treatment and prognosis There is no causal treatment. Osteotomies may be possible.[med-college.hu]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Here, we review the epidemiology, etiology, pathology, macrostructure, histology, and quantitative histomorphometry findings of PDB.[ncbi.nlm.nih.gov]
  • Abstract Although abundant evidence of concomitant cardiovascular disease is scattered throughout the literature on osteitis deformans, appreciation of the etiologic relationship between the two diseases has been lacking.[ahajournals.org]

Epidemiology

  • Here, we review the epidemiology, etiology, pathology, macrostructure, histology, and quantitative histomorphometry findings of PDB.[ncbi.nlm.nih.gov]
  • Imbalance between osteoclasts and osteoblasts Paget's Disease of Bone Epidemiology: What population does this affect? Arises in late adulthood (over 50) Paget's Disease of Bone Epidemiology: This arises in late adulthood (over 50).[quizlet.com]
  • Epidemiology Second most prevalent skeletal disease after osteoporosis Sex : (1.2:1) Age of onset : 55 years References: [1] [2] [3] Epidemiological data refers to the US, unless otherwise specified. Pathophysiology PDB is an idiopathic disease.[amboss.com]
Sex distribution
Age distribution

Pathophysiology

  • Although our understanding of the pathophysiology of PDB has advanced tremendously over the past 10 years, many questions remain unanswered, such as the mechanisms responsible for the focal nature of the disease and the recent changes in prevalence and[ncbi.nlm.nih.gov]
  • Pathophysiology and treatment of Paget's disease of bone, 2nd edn. London: Martin Dunitz, 1998. Rheumatology Vol. 44 No. 2 British Society for Rheumatology 2005; all rights reserved[doi.org]
  • *This picture above helps us in understanding the pathophysiology behind PAGET'S disease : *PATHOPHYSIOLOGY OF PAGET'S DISEASE: - The principal abnormality in Paget disease is the increased number and activity of osteoclasts. - Pagetic osteoclasts are[ourorthopaedics.blogspot.com]
  • Pathophysiology PDB is an idiopathic disease. PDB is associated with a high rate of bone remodeling.[amboss.com]

Prevention

  • Medical and surgical treatment is discussed with the recommendation that surgery is kept to a minimum and covered by prophylactic antibiotics to prevent osteomyelitis. Sudden enlargement of the involved bones should raise the suspicion of malignancy.[ncbi.nlm.nih.gov]
  • (This is to prevent hearing loss.) The calcium levels are elevated and causing symptoms. Drug therapy helps prevent further bone breakdown and formation. Currently, there are several classes of drugs used to treat Paget disease.[nlm.nih.gov]
  • Utilize isometric exercises to prevent muscle atrophy & to strengthen the muscle. Isometrics prevent joint from being immobilized. Position client with pillows to prevent strain on unaffected areas. Turn every 2 hours to prevent complications.[slideplayer.com]

References

Article

  1. Gordon G. Metabolic bone disease in practice. Am Pract Dig Treat. 1951;2:113.
  2. Clegg J. Paget's disease. Lancet. 1937;2:128.
  3. Ashworth C. Atherosclerotic valvular disease of the heart. Arch Path. 1946;42:285.
  4. Sutton D. Mitral stenosis. M Clin North Am. 1944;28: 47.
  5. Alvarez L, Guañabens N, Peris P, et al. Discriminative value of biochemical markers of bone turnover in assessing the activity of Paget's disease. J Bone Miner Res. 1995;10(3):458-65.
  6. Lluberas-Acosta G, Hansell JR, Schumacher HR Jr. Paget's disease of bone in patients with gout. Arch Intern Med. 1986;146(12):2389-92.
  7. Alexandersen P, Peris P, Guañabens N, et al. Non-isomerized C-telopeptide fragments are highly sensitive markers for monitoring disease activity and treatment efficacy in Paget's disease of bone. J Bone Miner Res. 2005;20(4):588-95.
  8. Drake MT, Clarke BL, Khosla S. Bisphosphonates: mechanism of action and role in clinical practice. Mayo Clin Proc. 2008;83(9):1032-45.
  9. Smith SE, Murphey MD, Motamedi K, Mulligan ME, Resnik CS, Gannon FH. From the archives of the AFIP. Radiologic spectrum of Paget disease of bone and its complications with pathologic correlation. Radiographics. 2002;22(5):1191-216.
  10. Seitz S, Priemel M, Zustin J, et al. Paget's disease of bone: histologic analysis of 754 patients. J Bone Miner Res. 2009;24(1):62-9.

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Last updated: 2019-07-11 21:25