Paget's disease of bone: Difference between revisions
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{{Infobox medical condition | {{Infobox medical condition | ||
| name | | name = Paget's disease of bone | ||
| image | | image = [[File:Pagets_skull.jpg|left|thumb|alt=Paget's disease of bone affecting the skull]] | ||
| caption | | caption = Paget's disease of bone affecting the skull | ||
| field | | field = [[Rheumatology]], [[Orthopedics]] | ||
| synonyms | | synonyms = Osteitis deformans | ||
| symptoms | | symptoms = Bone pain, deformities, fractures | ||
| complications | | complications = [[Osteosarcoma]], [[heart failure]], [[arthritis]] | ||
| onset | | onset = Usually after age 50 | ||
| duration | | duration = Long-term | ||
| causes | | causes = Unknown, possibly genetic and environmental factors | ||
| risks | | risks = Family history, age, [[European]] descent | ||
| diagnosis | | diagnosis = [[X-ray]], [[bone scan]], [[blood test]] for [[alkaline phosphatase]] | ||
| differential | | differential = [[Osteoporosis]], [[bone metastases]], [[fibrous dysplasia]] | ||
| treatment | | treatment = [[Bisphosphonates]], [[calcitonin]], [[pain management]] | ||
| prognosis | | prognosis = Generally good with treatment | ||
| frequency | | frequency = 1-2% of people over age 55 | ||
}} | }} | ||
Paget's disease is a disorder occurring in the middle-aged and elderly in which [[bone]] does not form properly, causing [[bone]] weakening, thickening, and deformity. | Paget's disease is a disorder occurring in the middle-aged and elderly in which [[bone]] does not form properly, causing [[bone]] weakening, thickening, and deformity.]] | ||
[[File:Paget's disease of Right Hip Bone.jpg|alt=Paget's disease of Right Hip Bone|left|thumb|Paget's disease of Right Hip Bone]] | [[File:Paget's disease of Right Hip Bone.jpg|left|thumb|alt=Paget's disease of Right Hip Bone|left|thumb|Paget's disease of Right Hip Bone]] | ||
==Diagnosis== | ==Diagnosis== | ||
Diagnosis is by X-rays and alkalinephosphatase blood test, and a bone scan. | Diagnosis is by X-rays and alkalinephosphatase blood test, and a bone scan.]] | ||
[[File:Paget's disease Wellcome L0003225.jpg|alt=Paget's disease|left|thumb|Paget's disease]] | [[File:Paget's disease Wellcome L0003225.jpg|left|thumb|alt=Paget's disease|left|thumb|Paget's disease]] | ||
==Pathophysiology== | ==Pathophysiology== | ||
Paget's disease of the bone represents an imbalance of bone formation and resorption. | Paget's disease of the bone represents an imbalance of bone formation and resorption. ]] | ||
[[File:Fémures de um indiv√≠duo com sexo e idade desconhecida. Fémur direito afetado pela doen√ßa de Paget.png|thumb|alt=Paget's disease of the femur|left|thumb|Paget's disease of the femur]] | |||
==Excess bone resorption== | ==Excess bone resorption== | ||
* It typically begins with excessive bone resorption followed by excessive bone formation. There is an exaggeration of osteoclastic bone resorption, initially producing a localized bone loss. | * It typically begins with excessive bone resorption followed by excessive bone formation. There is an exaggeration of osteoclastic bone resorption, initially producing a localized bone loss. | ||
* The excessive resorption and formation culminates at the tissue level in an abnormal mosaic pattern of lamellar bone associated with extensive vascularity and increased fibrous tissue deposition in adjacent marrow spaces. | * The excessive resorption and formation culminates at the tissue level in an abnormal mosaic pattern of lamellar bone associated with extensive vascularity and increased fibrous tissue deposition in adjacent marrow spaces. | ||
* Early in the disease process, osteolysis is accompanied by some level of repair. | * Early in the disease process, osteolysis is accompanied by some level of repair. | ||
* Paget's Disease can affect any bone in the body such as the spine, skull, pelvis, femora, and tibia are most commonly involved. | * Paget's Disease can affect any bone in the body such as the spine, skull, pelvis, femora, and tibia are most commonly involved. | ||
* Fractures as a result of this condition are common | * Fractures as a result of this condition are common]] | ||
[[File:Bone PagetDisease MP2 PA (2). | [[File:Bone PagetDisease MP2 PA (2).jpg|left|thumb|alt=Early Paget disease - cellular fibro-osseous lesion osteoclastic resorptive lacunae.|left|thumb|Early Paget's disease]] | ||
==Clinical presentation== | ==Clinical presentation== | ||
* Paget's Disease may occur in only one bone when it is called monostotic or in multiple bones when it is called polyostotic Paget's Disease. | * Paget's Disease may occur in only one bone when it is called monostotic or in multiple bones when it is called polyostotic Paget's Disease. | ||
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* The disorder can also lead to gross deformity, compression of roots or spinal cord, fracture of an involved bone, or alteration of joint structure and function leading to osteoarthritis | * The disorder can also lead to gross deformity, compression of roots or spinal cord, fracture of an involved bone, or alteration of joint structure and function leading to osteoarthritis | ||
* The overlying skin over the involved long bones may be warm and hyperemic, possibly due to increased blood flow to the entire region. | * The overlying skin over the involved long bones may be warm and hyperemic, possibly due to increased blood flow to the entire region. | ||
* Paget's Disease affecting the spine may produce pain directly or as a result of nerve root irritation, compression or both. | * Paget's Disease affecting the spine may produce pain directly or as a result of nerve root irritation, compression or both. ]] | ||
[[File:Paget's disease in vertebra on CT.jpg|alt=Paget's disease in vertebra on CT|left|thumb|Paget's disease in vertebra on CT]] | [[File:Paget's disease in vertebra on CT.jpg|left|thumb|alt=Paget's disease in vertebra on CT|left|thumb|Paget's disease in vertebra on CT]] | ||
==Nerve compression== | ==Nerve compression== | ||
* Compression of nerve fibers may also occur as well as involvement at the base of the skull may lead to basilar invagination or platybasia with consequent compression of structures in the posterior fossa, spinal cord or, rarely, cerebellar tonsillar herniation leading to ataxia, weakness, or respiratory compromise. | * Compression of nerve fibers may also occur as well as involvement at the base of the skull may lead to basilar invagination or platybasia with consequent compression of structures in the posterior fossa, spinal cord or, rarely, cerebellar tonsillar herniation leading to ataxia, weakness, or respiratory compromise. | ||
==Lack of strength in bone== | ==Lack of strength in bone== | ||
* The x-ray appearance of Pagetic bone reflects the underlying process | * The x-ray appearance of Pagetic bone reflects the underlying process | ||
* Pagetoid bone lacks the strength of normal bone and hence it deforms and fractures easily. | * Pagetoid bone lacks the strength of normal bone and hence it deforms and fractures easily. | ||
==Risk of cancer== | ==Risk of cancer== | ||
Patients with Paget's Disease may develop primary bone malignancies in the bone, including osteogenic carcinomas that can spread rapidly and have an extremely poor prognosis. | Patients with Paget's Disease may develop primary bone malignancies in the bone, including osteogenic carcinomas that can spread rapidly and have an extremely poor prognosis. | ||
{{stub}} | {{stub}} | ||
{{Osteochondropathy}} | {{Osteochondropathy}} | ||
{{ | {{Authority control}} | ||
{{DEFAULTSORT:Paget's Disease Of Bone}} | {{DEFAULTSORT:Paget's Disease Of Bone}} | ||
[[Category:Osteopathies]] | [[Category:Osteopathies]] | ||
[[Category:Osteitis]] | [[Category:Osteitis]] | ||
Revision as of 04:26, 14 April 2025
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD's weight loss doctor NYC
Philadelphia GLP-1 weight loss and GLP-1 clinic NYC
| Paget's disease of bone | |
|---|---|
| Synonyms | Osteitis deformans |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Bone pain, deformities, fractures |
| Complications | Osteosarcoma, heart failure, arthritis |
| Onset | Usually after age 50 |
| Duration | Long-term |
| Types | N/A |
| Causes | Unknown, possibly genetic and environmental factors |
| Risks | Family history, age, European descent |
| Diagnosis | X-ray, bone scan, blood test for alkaline phosphatase |
| Differential diagnosis | Osteoporosis, bone metastases, fibrous dysplasia |
| Prevention | N/A |
| Treatment | Bisphosphonates, calcitonin, pain management |
| Medication | N/A |
| Prognosis | Generally good with treatment |
| Frequency | 1-2% of people over age 55 |
| Deaths | N/A |
Paget's disease is a disorder occurring in the middle-aged and elderly in which bone does not form properly, causing bone weakening, thickening, and deformity.]]
Diagnosis
Diagnosis is by X-rays and alkalinephosphatase blood test, and a bone scan.]]
Pathophysiology
Paget's disease of the bone represents an imbalance of bone formation and resorption. ]]
Excess bone resorption
- It typically begins with excessive bone resorption followed by excessive bone formation. There is an exaggeration of osteoclastic bone resorption, initially producing a localized bone loss.
- The excessive resorption and formation culminates at the tissue level in an abnormal mosaic pattern of lamellar bone associated with extensive vascularity and increased fibrous tissue deposition in adjacent marrow spaces.
- Early in the disease process, osteolysis is accompanied by some level of repair.
- Paget's Disease can affect any bone in the body such as the spine, skull, pelvis, femora, and tibia are most commonly involved.
- Fractures as a result of this condition are common]]
Clinical presentation
- Paget's Disease may occur in only one bone when it is called monostotic or in multiple bones when it is called polyostotic Paget's Disease.
- The most common bones involved include spine, pelvis, skull, femur and tibia.
- It usually presents with pain
- Involvement of the skull and facial bones can result in leonine facies and sometimes loss of bone conduction hearing.
- The disorder can also lead to gross deformity, compression of roots or spinal cord, fracture of an involved bone, or alteration of joint structure and function leading to osteoarthritis
- The overlying skin over the involved long bones may be warm and hyperemic, possibly due to increased blood flow to the entire region.
- Paget's Disease affecting the spine may produce pain directly or as a result of nerve root irritation, compression or both. ]]
Nerve compression
- Compression of nerve fibers may also occur as well as involvement at the base of the skull may lead to basilar invagination or platybasia with consequent compression of structures in the posterior fossa, spinal cord or, rarely, cerebellar tonsillar herniation leading to ataxia, weakness, or respiratory compromise.
Lack of strength in bone
- The x-ray appearance of Pagetic bone reflects the underlying process
- Pagetoid bone lacks the strength of normal bone and hence it deforms and fractures easily.
Risk of cancer
Patients with Paget's Disease may develop primary bone malignancies in the bone, including osteogenic carcinomas that can spread rapidly and have an extremely poor prognosis.
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