Materiały źródłowe

• #1 Diagnosis and Treatment of Paget’s Disease of Bone | AAFP

  https://www.aafp.org/pubs/afp/issues/2002/0515/p2069.html

  Paget’s disease of bone (also known as osteitis deformans) is a nonmalignant disease involving accelerated bone resorption followed by deposition of dense, chaotic, and ineffectively mineralized bone matrix. […] Diagnosis is primarily made by radiographs. […] An estimated 70 percent of patients who have Paget’s disease have no symptoms. The diagnosis is typically found incidentally on radiographs and laboratory investigations. […] Diagnosis of Paget’s disease may be suspected based on the symptoms, but radiographs are the most specific diagnostic test. […] If the serum alkaline phosphatase level is elevated, a bone scan can be performed to determine the extent and activity of Paget’s disease. Radiographs should be taken to confirm the diagnosis in a patient with bone scans suggestive of Paget’s disease.

• #2 Paget’s disease of bone – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/525

  Pagets disease of bone (PDB) is a chronic localised bone remodelling disorder characterised by increased bone resorption, bone formation, and remodelling, which may lead to major long-bone and skull deformities. […] Diagnosis is incidental in the majority of patients, with an isolated elevated serum alkaline phosphatase raising suspicion for disease. Plain radiographs are diagnostic; in less clear cases, a computed tomography scan is indicated. Bone biopsy is the ultimate confirmatory test, but it is rarely necessary. […] Key diagnostic factors include family history of PDB and being asymptomatic. […] Other diagnostic factors include femoral, pelvis, and/or skull involvement, long-bone or back pain, pathological fracture, bony deformities (e.g., frontal bossing, prognathism, bone bowing), increased local temperature, hearing loss, facial pain, loosening teeth or disturbance in chewing, and isolated raised alkaline phosphatase.

• #3 Diagnosis and Treatment of Paget’s Disease of Bone | AAFP

  https://www.aafp.org/pubs/afp/issues/2002/0515/p2069.html

  Paget’s disease of bone (also known as osteitis deformans) is a nonmalignant disease involving accelerated bone resorption followed by deposition of dense, chaotic, and ineffectively mineralized bone matrix. […] Diagnosis is primarily made by radiographs. […] An estimated 70 percent of patients who have Paget’s disease have no symptoms. The diagnosis is typically found incidentally on radiographs and laboratory investigations. […] Diagnosis of Paget’s disease may be suspected based on the symptoms, but radiographs are the most specific diagnostic test. […] If the serum alkaline phosphatase level is elevated, a bone scan can be performed to determine the extent and activity of Paget’s disease. Radiographs should be taken to confirm the diagnosis in a patient with bone scans suggestive of Paget’s disease.

• #4 Paget’s Disease of Bone – OrthoInfo – AAOS

  https://orthoinfo.aaos.org/en/diseases–conditions/pagets-disease-of-bone

  Paget’s disease of bone is a chronic disease of the skeleton. In healthy bone, a process called remodeling removes old pieces of bone and replaces them with new, fresh bone. Pagets disease causes this process to shift out of balance, resulting in new bone that is abnormally shaped, weak, and brittle. Pagets disease most often affects older people, occurring in approximately 2 to 3% of the population over the age of 55. […] Many patients with Pagets disease have no symptoms at all and are unaware they have the disease until X-rays are taken for some other reason. When bone pain and other symptoms are present, they can be related to the disease itself or to complications that arise from the disease such as arthritis, bone deformity, and fractures. […] In many cases, Pagets disease is discovered incidentally, when X-rays are taken or bloodwork is performed for some other reason. If this occurs, your doctor will perform a physical examination and order a number of tests to confirm the diagnosis.

• #5 Paget’s Disease of Bone

  https://rheumatology.org/patients/pagets-disease-of-bone

  Symptoms of Pagets disease include joint and bone pain, headaches, hearing loss, enlarged bones, bowed arms or legs, weak or soft bones that bend or break, tingling, and numbness. […] Certain blood and urine tests can raise suspicion for Pagets disease and may be suspected and confirmed by imaging studies such as x-rays showing abnormal bone structure. […] Usually following diagnosis, non-invasive bone scans may be used to show the extent of bone involvement. […] A biopsy is used only if cancer is suspected which occurs less, which should be of concern in all cases but rarely occurs. […] Prompt diagnosis and treatment can help them manage their disease effectively.

• #6 Paget’s disease of bone – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/525

  Pagets disease of bone (PDB) is a chronic localised bone remodelling disorder characterised by increased bone resorption, bone formation, and remodelling, which may lead to major long-bone and skull deformities. […] Diagnosis is incidental in the majority of patients, with an isolated elevated serum alkaline phosphatase raising suspicion for disease. Plain radiographs are diagnostic; in less clear cases, a computed tomography scan is indicated. Bone biopsy is the ultimate confirmatory test, but it is rarely necessary. […] Key diagnostic factors include family history of PDB and being asymptomatic. […] Other diagnostic factors include femoral, pelvis, and/or skull involvement, long-bone or back pain, pathological fracture, bony deformities (e.g., frontal bossing, prognathism, bone bowing), increased local temperature, hearing loss, facial pain, loosening teeth or disturbance in chewing, and isolated raised alkaline phosphatase.

• #7 Paget’s disease of bone – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-bone/diagnosis-treatment/drc-20350816

  During the physical exam, your doctor will examine areas of your body that are causing you pain. He or she may also order X-rays and blood tests that can help confirm the diagnosis of Paget’s disease of bone. […] The first indication of Paget’s disease of bone is often abnormalities found on X-rays done for other reasons. X-ray images of your bones can show areas of bone breakdown, enlargement of the bone and deformities that are characteristic of the disease, such as bowing of your long bones. […] People who have Paget’s disease of bone usually have elevated levels of alkaline phosphatase in their blood, which can be revealed by a blood test. […] Most people with Paget’s disease of bone don’t have any symptoms and are diagnosed when an X-ray or blood test taken for another reason reveals signs of Paget’s disease of bone.

• #8 Paget’s disease of bone – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/525

  Pagets disease of bone (PDB) is a chronic localised bone remodelling disorder characterised by increased bone resorption, bone formation, and remodelling, which may lead to major long-bone and skull deformities. […] Diagnosis is incidental in the majority of patients, with an isolated elevated serum alkaline phosphatase raising suspicion for disease. Plain radiographs are diagnostic; in less clear cases, a computed tomography scan is indicated. Bone biopsy is the ultimate confirmatory test, but it is rarely necessary. […] Key diagnostic factors include family history of PDB and being asymptomatic. […] Other diagnostic factors include femoral, pelvis, and/or skull involvement, long-bone or back pain, pathological fracture, bony deformities (e.g., frontal bossing, prognathism, bone bowing), increased local temperature, hearing loss, facial pain, loosening teeth or disturbance in chewing, and isolated raised alkaline phosphatase.

• #9 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Diagnosis-of-Pagets-disease-of-the-bone.aspx

  Diagnosis of this condition is usually made in individuals over 40. It is rare in younger individuals. […] Diagnosis of the condition may involve X-rays, blood tests and so forth. […] In some patients there may be no symptoms of the condition. Diagnosis in them is made by chance on X ray of the affected joint or on routine blood tests. […] Blood tests include those for serum alkaline phosphatase. This enzyme measures the bone turnover. It is usually quite elevated in patients with Paget’s disease. […] Urine tests also show signs of excess Calcium and rapid bone turnover. Urine levels of deoxypyridinoline and N-telopeptide are elevated. […] A bone scan or a PET (Positron Emission tomography) scan is advised for patients with Pagets disease. […] Bone samples may sometimes be needed for biopsy. It is important to confirm the diagnosis of Pagets disease. […] Hearing loss may be the first to be detected in Pagets disease affecting the skull bones. […] Due to high blood levels of Calcium from increased bone breakdown kidney stones may occur. These are detected by imaging studies like Ultrasound and X rays.

• #10 Paget’s disease of bone – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-bone/diagnosis-treatment/drc-20350816

  During the physical exam, your doctor will examine areas of your body that are causing you pain. He or she may also order X-rays and blood tests that can help confirm the diagnosis of Paget’s disease of bone. […] The first indication of Paget’s disease of bone is often abnormalities found on X-rays done for other reasons. X-ray images of your bones can show areas of bone breakdown, enlargement of the bone and deformities that are characteristic of the disease, such as bowing of your long bones. […] People who have Paget’s disease of bone usually have elevated levels of alkaline phosphatase in their blood, which can be revealed by a blood test. […] Most people with Paget’s disease of bone don’t have any symptoms and are diagnosed when an X-ray or blood test taken for another reason reveals signs of Paget’s disease of bone.

• #11 Paget’s Disease of Bone

  https://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/pagets-disease-of-bone/

  Thus, bone scans are particularly useful when a patient first presents with symptoms of PDB. As soon as abnormalities are identified by bone scan, they should be confirmed by conventional radiography in at least one site of bone. […] Bone biopsy may be necessary if malignant transformation of the bone is suspected. The most important feature of malignant change of the bone is cortical destruction and the presence of a soft tissue mass outside of the bone. […] PDB is characterized by accelerated bone turnover. Therefore, elevated levels of biochemical markers of bone turnover provide a general indication of the extent of disease activity. Total serum alkaline phosphatase activity, which is a reflection of increased bone formation, is elevated in 85% of untreated PDB patients. […] In studies that compare various markers of bone turnover in patients with PDB, bone-specific alkaline phosphatase has a diagnostic sensitivity of 84%, and total serum alkaline phosphatase has a diagnostic sensitivity of 74%. […] The alkaline phosphatase is a useful marker of disease activity, extent and severity of disease, and treatment efficacy. Accelerated bone resorption also occurs in PDB. Thus, increased urinary excretion of pyridinoline and hydroxyproline, markers of bone resorption, are observed.

• #12 Diagnosis | Paget’s Association

  https://paget.org.uk/pagets-disease/what-is-pagets-and-how-is-it-managed/diagnosis/

  It has been estimated that less than 10% of patients with x-ray evidence of Pagets disease come to medical attention. […] If there is overactivity of the osteoblasts due to Pagets disease, alkaline phosphatase is released into the bloodstream and can be measured. […] When Pagets disease is active, the ALP level will often, but not always, be raised. […] A radionuclide bone scan is the best way of fully evaluating the extent of Pagets disease. […] A scan is recommended to determine which bones have Pagets disease and how active the disease is.

• #13 Paget’s Disease: Causes, Symptoms, and Treatment | Doctor

  https://patient.info/doctor/pagets-disease-of-bone-pro

  Most cases of PDB are diagnosed incidentally on X-rays or as an isolated elevation of serum alkaline phosphatase. Symptomatic patients present with bone pain, deformity, fractures, arthritis and features of compression neuropathy. […] Serum total alkaline phosphatase is recommended as a first-line biochemical screening test in combination with liver function tests in screening for the presence of metabolically active Paget’s disease of bone. […] Radionuclide bone scans, in addition to targeted X-rays, are recommended as a means of fully and accurately defining the extent of metabolically active disease. […] Bone biopsy may be needed if malignant change is suspected. […] The objectives of treatment are control of pain and to reduce or prevent disease progression and complications. […] Referral to secondary care is advisable in a patient thought to have pain or deformity so that further assessment can be performed and treatment offered if appropriate. […] Serial monitoring of alkaline phosphatase is used to monitor the effects of treatment and disease activity.

• #14 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Measurement of serum alkaline phosphatase in some cases, bone-specific alkaline phosphatase (BSAP) can be useful in the diagnosis of Paget disease. Elevated levels of urinary markers, including hydroxyproline, deoxypyridinoline, C-telopeptide, and N-telopeptide, may help identify patients with Paget disease. […] Procollagen I N-terminal peptide (PINP) has emerged as a sensitive serum marker for bone formation. Serum osteocalcin, which is produced specifically by osteoblasts, does not reflect disease activity. […] Serum calcium and phosphate levels should be within the reference range in patients with Paget disease. Urinary excretion of calcium also should be normal. However, hypercalcemia or hypercalciuria may develop with immobilization or coincident primary hyperparathyroidism. […] Plain radiographs and bone scanning should be performed upon initial diagnosis. Radiography is typically used for diagnostic purposes.

• #15 Treatment of Paget’s Disease of Bone

  https://www.uspharmacist.com/article/treatment-of-pagets-disease-of-bone

  Since Pagets disease often is asymptomatic, it frequently is discovered accidentally because of an elevated serum alkaline phosphatase (ALK-P) of unknown origin or upon radiography performed to evaluate abdominal symptoms or bone, pelvic, or hip pain. Ninety-five percent of untreated patients with Pagets disease have an elevated serum ALK-P level; however, a normal concentration does not exclude the diagnosis. Serum ALK-P may be normal in patients with monostotic or metabolically inactive disease. Serum ALK-P elevations are reflective of an increased number of osteoblasts in the lesions. The level is indicative of both disease extent and disease activity. Serum total ALK-P levels are appropriate for following the disease, but the bone-specific isoform should be used in patients with coexisting hepatobiliary disease.

• #16 Paget Disease: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/334607-overview

  Paget disease is a localized disorder of bone remodeling that typically begins with excessive bone resorption followed by an increase in bone formation. […] Measurement of serum alkaline phosphatase in some cases, bone-specific alkaline phosphatase (BSAP) along with several urinary markers, can be useful in the diagnosis of Paget disease. Plain radiographs and bone scanning should be performed upon initial diagnosis. […] The degree of elevation of these biomarkers helps identify the extent and severity of bone turnover. […] Markers of bone turnover that are useful to monitor in persons with Paget disease include the following: Bone-specific alkaline phosphatase (marker of bone formation), Deoxypyridinoline (marker of bone resorption), N -Telopeptide of type I collagen (marker of bone resorption), Alpha-alpha type I C-telopeptide fragments. […] Upon successful treatment of Paget disease, the level of these bone markers is expected to decrease.

• #17 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Measurement of serum alkaline phosphatase in some cases, bone-specific alkaline phosphatase (BSAP) can be useful in the diagnosis of Paget disease. Elevated levels of urinary markers, including hydroxyproline, deoxypyridinoline, C-telopeptide, and N-telopeptide, may help identify patients with Paget disease. […] Procollagen I N-terminal peptide (PINP) has emerged as a sensitive serum marker for bone formation. Serum osteocalcin, which is produced specifically by osteoblasts, does not reflect disease activity. […] Serum calcium and phosphate levels should be within the reference range in patients with Paget disease. Urinary excretion of calcium also should be normal. However, hypercalcemia or hypercalciuria may develop with immobilization or coincident primary hyperparathyroidism. […] Plain radiographs and bone scanning should be performed upon initial diagnosis. Radiography is typically used for diagnostic purposes.

• #18 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Measurement of serum alkaline phosphatase in some cases, bone-specific alkaline phosphatase (BSAP) can be useful in the diagnosis of Paget disease. Elevated levels of urinary markers, including hydroxyproline, deoxypyridinoline, C-telopeptide, and N-telopeptide, may help identify patients with Paget disease. […] Procollagen I N-terminal peptide (PINP) has emerged as a sensitive serum marker for bone formation. Serum osteocalcin, which is produced specifically by osteoblasts, does not reflect disease activity. […] Serum calcium and phosphate levels should be within the reference range in patients with Paget disease. Urinary excretion of calcium also should be normal. However, hypercalcemia or hypercalciuria may develop with immobilization or coincident primary hyperparathyroidism. […] Plain radiographs and bone scanning should be performed upon initial diagnosis. Radiography is typically used for diagnostic purposes.

• #19 SciELO Brasil – Diagnosis and treatment of Paget’s disease of bone: a mini-review Diagnosis and treatment of Paget’s disease of bone: a mini-review

  https://www.scielo.br/j/abem/a/g9kNBjYfcqFynGtTdtTGsxg/

  The laboratory hallmark of PDB is increased serum ALP levels, mainly due to the extensive experience with the use of this easily obtainable bone formation marker. […] Plain radiographies of affected bones and bone scintigraphy are most frequently used in diagnosing PDB. […] PDB should be differentiated from rarer disorders, such as fibrous dysplasia of bone, which normally affects individuals under 40 years of age, or hyperostosis frontalis interna, a benign affection of the skull. […] Antiresorptive therapy is the mainstay of PDB medical treatment, and its main objective is to achieve symptom relief, namely bone pain. […] Bisphosphonates are potent antiresorptive agents with high-affinity for bone and, thus, very effective in reducing metabolic activity and achieving symptomatic control in PDB.

• #20 Paget’s disease of bone – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/pagets-disease-of-bone/diagnosis-treatment/drc-20350816

  During the physical exam, your doctor will examine areas of your body that are causing you pain. He or she may also order X-rays and blood tests that can help confirm the diagnosis of Paget’s disease of bone. […] The first indication of Paget’s disease of bone is often abnormalities found on X-rays done for other reasons. X-ray images of your bones can show areas of bone breakdown, enlargement of the bone and deformities that are characteristic of the disease, such as bowing of your long bones. […] People who have Paget’s disease of bone usually have elevated levels of alkaline phosphatase in their blood, which can be revealed by a blood test. […] Most people with Paget’s disease of bone don’t have any symptoms and are diagnosed when an X-ray or blood test taken for another reason reveals signs of Paget’s disease of bone.

• #21 Diagnosis and Treatment of Paget’s Disease of Bone | AAFP

  https://www.aafp.org/pubs/afp/issues/2002/0515/p2069.html

  Paget’s disease of bone (also known as osteitis deformans) is a nonmalignant disease involving accelerated bone resorption followed by deposition of dense, chaotic, and ineffectively mineralized bone matrix. […] Diagnosis is primarily made by radiographs. […] An estimated 70 percent of patients who have Paget’s disease have no symptoms. The diagnosis is typically found incidentally on radiographs and laboratory investigations. […] Diagnosis of Paget’s disease may be suspected based on the symptoms, but radiographs are the most specific diagnostic test. […] If the serum alkaline phosphatase level is elevated, a bone scan can be performed to determine the extent and activity of Paget’s disease. Radiographs should be taken to confirm the diagnosis in a patient with bone scans suggestive of Paget’s disease.

• #22 Paget’s Disease of Bone – OrthoInfo – AAOS

  https://orthoinfo.aaos.org/en/diseases–conditions/pagets-disease-of-bone

  X-rays provide images of dense structures, such as bone. Pagets disease can usually be diagnosed by looking at an X-ray. A bone affected by Pagets usually appears larger and denser than a normal bone. It may also have a deformed shape. […] A blood test called serum alkaline phosphatase may also be used to help confirm the diagnosis. In patients with Pagets disease, alkaline phosphatase levels are usually quite elevated a reflection of the high bone turnover rate. […] Paget’s disease can also be detected with urine tests that show rapid bone turnover. […] A biopsy is sometimes necessary to confirm the diagnosis of Paget’s disease or to rule out other conditions.

• #23 Paget disease (bone) | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/paget-disease-bone?lang=us

  Paget disease of the bone is a common, chronic metabolic bone disorder characterized by excessive abnormal bone remodeling. The classically described radiological appearances are expanded bone with a coarsened trabecular pattern. The pelvis, spine, skull, and proximal long bones are most frequently affected. […] The majority (approximately three-quarters) of patients are asymptomatic at the time of diagnosis, the diagnosis being an incidental finding on imaging. Presenting symptoms include localized pain and tenderness (most common symptom), increased focal temperature due to bony hypervascularity (not fever), increased bone size, bowing deformities, kyphosis, decreased range of motion, and signs and symptoms relating to complications. […] The early phase features osteolytic (lucent) regions which are later followed by coarsened trabeculae and bony enlargement. Sclerotic changes occur much later in the disease process.

• #24 Paget disease (bone) | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/paget-disease-bone?lang=us

  Paget disease of the bone is a common, chronic metabolic bone disorder characterized by excessive abnormal bone remodeling. The classically described radiological appearances are expanded bone with a coarsened trabecular pattern. The pelvis, spine, skull, and proximal long bones are most frequently affected. […] The majority (approximately three-quarters) of patients are asymptomatic at the time of diagnosis, the diagnosis being an incidental finding on imaging. Presenting symptoms include localized pain and tenderness (most common symptom), increased focal temperature due to bony hypervascularity (not fever), increased bone size, bowing deformities, kyphosis, decreased range of motion, and signs and symptoms relating to complications. […] The early phase features osteolytic (lucent) regions which are later followed by coarsened trabeculae and bony enlargement. Sclerotic changes occur much later in the disease process.

• #25 Paget disease of bone An update

  https://www.racgp.org.au/afp/2012/march/paget-disease-of-bone

  The classic presentation of Paget disease (as described by Sir James Paget in 1877) as a painful, deforming disease is now uncommon. Nowadays, PDB is detected most frequently as an incidental finding on radiographs or on biochemistry (as raised alkaline phosphatase activity). […] Paget disease of the bone is diagnosed radiologically. Early in the course of the disease, lytic activity predominates, causing focal osteolytic lesions (osteoporosis circumscripta) or flame shaped, advancing lytic wedges in the long bones. […] Isotope bone scanning is more sensitive than plain radiology in detecting pagetic lesions, and is indicated in most newly diagnosed patients to determine the distribution of the disease. This allows the identification of involved bones with the potential for complications, such as the base of the skull, spine and long bones.

• #26 Paget’s Disease of Bone: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/pagets-disease-bone/diagnosis-treatment-and-steps-to-take

  Many people with Pagets disease are diagnosed after having tests for other conditions or to help diagnose the cause for their pain. Some common tests used to diagnose Pagets disease include: […] This is the most common test that doctors use to diagnose the disease. An x-ray can evaluate the bone structure for Pagets disease. […] Your doctor may order a blood test to check for the enzyme alkaline phosphatase. This test can indicate either Pagets disease or another condition such as liver disease. If your blood contains high levels of the enzyme, it may be a sign of the disease. […] A bone scan is a test that helps doctors identify which bones are affected by Pagets disease. The test may help your doctor understand the extent of the disease. During a bone scan, a safe amount of radioactive substance is injected into a vein in your arm. The substance circulates through the bloodstream and highlights places in your skeleton where Pagets disease may be present by collecting in areas where there is increased blood flow and activity of bone-forming cells characteristic of Pagets disease.

• #27 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Plain radiographs are less sensitive than bone scan scintigraphy in the diagnosis of Paget disease. […] Computed tomography (CT) scanning and magnetic resonance imaging (MRI) are not needed for the diagnosis of Paget disease of bone. However, both are useful in the evaluation of complications of Paget disease, such as neoplastic degeneration, articular abnormalities, and spinal involvement with neurologic compromise. […] Bone scanning is the most sensitive test for evaluating the extent of lesions in the whole skeleton affected by Paget disease. Bone scintigraphic abnormalities are observed earlier than radiographic changes during the active stage of Paget disease. […] Articular abnormalities require CT scanning or MRI to delineate the extent of involvement. CT scanning and MRI are useful to diagnose and evaluate neurologic complications, such as basilar invagination, spinal cord compression, or hydrocephalus. […] The major histologic feature of Paget disease is abnormal bony architecture. The 3 distinct phases in Paget disease (osteolytic, mixed, and osteosclerotic) may exist separately or in the same bone at one time.

• #28 Paget’s Disease of Bone: Diagnosis, Treatment, and Steps to Take

  https://www.niams.nih.gov/health-topics/pagets-disease-bone/diagnosis-treatment-and-steps-to-take

  Many people with Pagets disease are diagnosed after having tests for other conditions or to help diagnose the cause for their pain. Some common tests used to diagnose Pagets disease include: […] This is the most common test that doctors use to diagnose the disease. An x-ray can evaluate the bone structure for Pagets disease. […] Your doctor may order a blood test to check for the enzyme alkaline phosphatase. This test can indicate either Pagets disease or another condition such as liver disease. If your blood contains high levels of the enzyme, it may be a sign of the disease. […] A bone scan is a test that helps doctors identify which bones are affected by Pagets disease. The test may help your doctor understand the extent of the disease. During a bone scan, a safe amount of radioactive substance is injected into a vein in your arm. The substance circulates through the bloodstream and highlights places in your skeleton where Pagets disease may be present by collecting in areas where there is increased blood flow and activity of bone-forming cells characteristic of Pagets disease.

• #29 Paget’s Disease of Bone

  https://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/rheumatology/pagets-disease-of-bone/

  Thus, bone scans are particularly useful when a patient first presents with symptoms of PDB. As soon as abnormalities are identified by bone scan, they should be confirmed by conventional radiography in at least one site of bone. […] Bone biopsy may be necessary if malignant transformation of the bone is suspected. The most important feature of malignant change of the bone is cortical destruction and the presence of a soft tissue mass outside of the bone. […] PDB is characterized by accelerated bone turnover. Therefore, elevated levels of biochemical markers of bone turnover provide a general indication of the extent of disease activity. Total serum alkaline phosphatase activity, which is a reflection of increased bone formation, is elevated in 85% of untreated PDB patients. […] In studies that compare various markers of bone turnover in patients with PDB, bone-specific alkaline phosphatase has a diagnostic sensitivity of 84%, and total serum alkaline phosphatase has a diagnostic sensitivity of 74%. […] The alkaline phosphatase is a useful marker of disease activity, extent and severity of disease, and treatment efficacy. Accelerated bone resorption also occurs in PDB. Thus, increased urinary excretion of pyridinoline and hydroxyproline, markers of bone resorption, are observed.

• #30 Paget’s Disease: Causes, Symptoms, and Treatment | Doctor

  https://patient.info/doctor/pagets-disease-of-bone-pro

  Most cases of PDB are diagnosed incidentally on X-rays or as an isolated elevation of serum alkaline phosphatase. Symptomatic patients present with bone pain, deformity, fractures, arthritis and features of compression neuropathy. […] Serum total alkaline phosphatase is recommended as a first-line biochemical screening test in combination with liver function tests in screening for the presence of metabolically active Paget’s disease of bone. […] Radionuclide bone scans, in addition to targeted X-rays, are recommended as a means of fully and accurately defining the extent of metabolically active disease. […] Bone biopsy may be needed if malignant change is suspected. […] The objectives of treatment are control of pain and to reduce or prevent disease progression and complications. […] Referral to secondary care is advisable in a patient thought to have pain or deformity so that further assessment can be performed and treatment offered if appropriate. […] Serial monitoring of alkaline phosphatase is used to monitor the effects of treatment and disease activity.

• #31 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Plain radiographs are less sensitive than bone scan scintigraphy in the diagnosis of Paget disease. […] Computed tomography (CT) scanning and magnetic resonance imaging (MRI) are not needed for the diagnosis of Paget disease of bone. However, both are useful in the evaluation of complications of Paget disease, such as neoplastic degeneration, articular abnormalities, and spinal involvement with neurologic compromise. […] Bone scanning is the most sensitive test for evaluating the extent of lesions in the whole skeleton affected by Paget disease. Bone scintigraphic abnormalities are observed earlier than radiographic changes during the active stage of Paget disease. […] Articular abnormalities require CT scanning or MRI to delineate the extent of involvement. CT scanning and MRI are useful to diagnose and evaluate neurologic complications, such as basilar invagination, spinal cord compression, or hydrocephalus. […] The major histologic feature of Paget disease is abnormal bony architecture. The 3 distinct phases in Paget disease (osteolytic, mixed, and osteosclerotic) may exist separately or in the same bone at one time.

• #32 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Plain radiographs are less sensitive than bone scan scintigraphy in the diagnosis of Paget disease. […] Computed tomography (CT) scanning and magnetic resonance imaging (MRI) are not needed for the diagnosis of Paget disease of bone. However, both are useful in the evaluation of complications of Paget disease, such as neoplastic degeneration, articular abnormalities, and spinal involvement with neurologic compromise. […] Bone scanning is the most sensitive test for evaluating the extent of lesions in the whole skeleton affected by Paget disease. Bone scintigraphic abnormalities are observed earlier than radiographic changes during the active stage of Paget disease. […] Articular abnormalities require CT scanning or MRI to delineate the extent of involvement. CT scanning and MRI are useful to diagnose and evaluate neurologic complications, such as basilar invagination, spinal cord compression, or hydrocephalus. […] The major histologic feature of Paget disease is abnormal bony architecture. The 3 distinct phases in Paget disease (osteolytic, mixed, and osteosclerotic) may exist separately or in the same bone at one time.

• #33 Paget’s Disease of Bone: Symptoms, Diagnosis, Treatment

  https://www.webmd.com/osteoporosis/pagets-disease-bone

  Bone scan: A small amount of a radioactive substance, called a tracer, is put into a vein in your arm. It goes through your bloodstream and into your bones. A special camera takes pictures of your bones and any areas that absorb too much or too little of the tracer can be a sign of a problem with the bone. A bone scan is somewhat better than xray for diagnosis, especially early on. It also shows the extent of paget lesions in your bones cause it is not targeted at specific bones (like an xray). […] MRI (magnetic resonance imaging): Powerful magnets and radio waves are used to make detailed images of your bones. It is also helpful in determining if there is osteosarcoma or a bone malignancy which Pagets put you at risk for. […] CT (computerized tomography) scan: Several X-rays are taken from different angles and put together to make a more complete picture of your bones. It, too, can detect osteosarcoma.

• #34 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Plain radiographs are less sensitive than bone scan scintigraphy in the diagnosis of Paget disease. […] Computed tomography (CT) scanning and magnetic resonance imaging (MRI) are not needed for the diagnosis of Paget disease of bone. However, both are useful in the evaluation of complications of Paget disease, such as neoplastic degeneration, articular abnormalities, and spinal involvement with neurologic compromise. […] Bone scanning is the most sensitive test for evaluating the extent of lesions in the whole skeleton affected by Paget disease. Bone scintigraphic abnormalities are observed earlier than radiographic changes during the active stage of Paget disease. […] Articular abnormalities require CT scanning or MRI to delineate the extent of involvement. CT scanning and MRI are useful to diagnose and evaluate neurologic complications, such as basilar invagination, spinal cord compression, or hydrocephalus. […] The major histologic feature of Paget disease is abnormal bony architecture. The 3 distinct phases in Paget disease (osteolytic, mixed, and osteosclerotic) may exist separately or in the same bone at one time.

• #35 Diagnosis and Treatment of Paget’s Disease of Bone | AAFP

  https://www.aafp.org/pubs/afp/issues/2002/0515/p2069.html

  There are many biochemical markers for Paget’s disease, but the two most important are total serum alkaline phosphatase and urinary pyridinoline. […] Many patients are diagnosed incidentally in the asymptomatic phase by plain radiographs that show localized enlargement of bone. […] Once a diagnosis of Paget’s disease is confirmed, repeat radiographs are required only to monitor degeneration around weight-bearing joints. […] If the results of the biochemical markers and radiography are inconclusive, a biopsy of the affected bone may be indicated in rare cases.

• #36 Paget’s Disease of Bone Causes, Symptoms, Treatment, Medications, Prevention

  https://www.medicinenet.com/pagets_disease/article.htm

  Paget’s disease is a chronic condition of bone characterized by disorder of the normal bone remodeling process. […] How is Paget’s disease diagnosed? […] Health care professionals make a diagnosis of Paget’s disease based on the X-ray appearance. Doctors also may detect Paget’s disease with other imaging tests, such as a bone scan, MRI scan, and CT scan to make a diagnosis. […] The bone scan is particularly helpful in determining the extent of the involvement of Paget’s disease as it provides an image of the entire skeleton. Bone that is affected by Paget’s disease can easily be identified with bone scanning images. […] A bone biopsy is generally not necessary to make the diagnosis of Paget’s disease. Bone biopsy would be considered if there was a suspicion of cancer or abnormal lesion in the involved bone.

• #37 Diagnosis | Osteitis deformans or Paget’s disease of bone | Lab Animal

  https://www.nature.com/articles/laban1008-450

  We believe that in our rat this diagnosis can be validated by simple clinical inspection, given the deformities observed in the skull. […] But the pathognomonic feature of osteitis deformans must be seen on histology. It has been described as a tile-like or 'mosaic’ pattern observed in both the cortical and cancellous regions of the affected bones, which accounts for the softening, porosity and subperiosteal new bone formation and contributes to the abnormal structural integrity. Numerous osteoblasts and osteoclasts are present and have been interpreted as exaggerated functional activity.

• #38 Diagnosis | Osteitis deformans or Paget’s disease of bone | Lab Animal

  https://www.nature.com/articles/laban1008-450

  We believe that in our rat this diagnosis can be validated by simple clinical inspection, given the deformities observed in the skull. […] But the pathognomonic feature of osteitis deformans must be seen on histology. It has been described as a tile-like or 'mosaic’ pattern observed in both the cortical and cancellous regions of the affected bones, which accounts for the softening, porosity and subperiosteal new bone formation and contributes to the abnormal structural integrity. Numerous osteoblasts and osteoclasts are present and have been interpreted as exaggerated functional activity.

• #39 Paget Disease of Bone for Primary Care | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p224.html

  Paget disease of bone may be asymptomatic, and suspicion arises from incidental findings of elevated serum alkaline phosphatase levels on routine blood work or abnormalities on imaging tests performed for an unrelated cause. […] Evidence-based guidelines recommend the use of plain radiography and serum alkaline phosphatase testing for initial diagnosis and radionuclide scans for delineation of the extent of disease. […] Diagnosis requires a high index of suspicion because many symptoms of Paget disease of bone are nonspecific. Clinical history should explore skeletal pain to establish a probable source; for example, it is imperative to assess whether the fractures are the result of significant trauma. Family history of Paget disease of bone should be assessed because a genetic association may serve as a supporting diagnostic clue. The family physician may need to consult with a specialist for confirmation of diagnosis and for initiation of treatment.

• #40 Paget’s disease of bone – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/525

  Pagets disease of bone (PDB) is a chronic localised bone remodelling disorder characterised by increased bone resorption, bone formation, and remodelling, which may lead to major long-bone and skull deformities. […] Diagnosis is incidental in the majority of patients, with an isolated elevated serum alkaline phosphatase raising suspicion for disease. Plain radiographs are diagnostic; in less clear cases, a computed tomography scan is indicated. Bone biopsy is the ultimate confirmatory test, but it is rarely necessary. […] Key diagnostic factors include family history of PDB and being asymptomatic. […] Other diagnostic factors include femoral, pelvis, and/or skull involvement, long-bone or back pain, pathological fracture, bony deformities (e.g., frontal bossing, prognathism, bone bowing), increased local temperature, hearing loss, facial pain, loosening teeth or disturbance in chewing, and isolated raised alkaline phosphatase.

• #41 Paget’s disease of bone – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/525

  1st investigations to order include plain x-ray, bone scan, total serum alkaline phosphatase, bone-specific alkaline phosphatase, serum calcium, serum procollagen 1 N-terminal peptide (P1NP), serum C-terminal propeptide of type 1 collagen (CTX), liver function tests, and serum 25-hydroxyvitamin D. […] Investigations to consider include CT scan or MRI and bone biopsy.

• #42 Paget’s disease of bone: when and why to refer to specialist care | British Journal of General Practice

  https://bjgp.org/content/70/700/561

  Pagets disease of bone (PDB) is characterised by increased but disorganised bone remodelling, leading to various complications including pain, deformity, and fracture. […] This article provides guidance to primary care teams on the diagnosis and management of patients with PDB based on a recent clinical guideline. Patients with an isolated elevation in ALP should be considered for radionuclide bone scan imaging as this is the most sensitive way of detecting PDB. […] If bone scans are not readily available, then plain X-rays of the abdomen (including the ribs and femoral heads), both tibias, the skull, and facial bones may be considered since X-rays of these sites have been found to detect PDB in 90% of patients. […] The key investigation is ALP, which gives an indication if PDB is metabolically active, but liver function tests, calcium, phosphate, vitamin D, and creatinine are helpful in the differential diagnosis from other conditions such as renal osteodystrophy, primary hyperparathyroidism, and osteomalacia.

• #43 Paget Disease of Bone for Primary Care | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p224.html

  All patients who are thought to have Paget disease of bone should have plain radiography of the suspected sites. […] In asymptomatic patients with elevated levels of total serum alkaline phosphatase and no underlying liver or biliary tract disease, plain radiographs of the abdomen, skull and facial bones, and tibia should be obtained. […] Patients diagnosed with Paget disease of bone should have a radionuclide bone scan to fully assess the extent of the disease. […] Measurement of alkaline phosphatase in conjunction with liver function testing is used as a first-line biochemical evaluation. In suspected cases of Paget disease of bone in which total serum alkaline phosphatase levels are normal or hepatic disease exists, measurement of specific bone markers such as procollagen type I N-terminal propeptide, bone-specific alkaline phosphatase, or urinary cross-linked N-terminal telopeptide of type I collagen is indicated.

• #44 Paget’s disease of bone: when and why to refer to specialist care | British Journal of General Practice

  https://bjgp.org/content/70/700/561

  Pagets disease of bone (PDB) is characterised by increased but disorganised bone remodelling, leading to various complications including pain, deformity, and fracture. […] This article provides guidance to primary care teams on the diagnosis and management of patients with PDB based on a recent clinical guideline. Patients with an isolated elevation in ALP should be considered for radionuclide bone scan imaging as this is the most sensitive way of detecting PDB. […] If bone scans are not readily available, then plain X-rays of the abdomen (including the ribs and femoral heads), both tibias, the skull, and facial bones may be considered since X-rays of these sites have been found to detect PDB in 90% of patients. […] The key investigation is ALP, which gives an indication if PDB is metabolically active, but liver function tests, calcium, phosphate, vitamin D, and creatinine are helpful in the differential diagnosis from other conditions such as renal osteodystrophy, primary hyperparathyroidism, and osteomalacia.

• #45 Paget’s Disease of Bone | Endocrine Society

  https://www.endocrine.org/clinical-practice-guidelines/pagets-disease-of-bone

  The aim of this guideline was to formulate practice guidelines for the diagnosis and treatment of Paget’s disease of the bone. […] We recommend that plain radiographs be obtained of the pertinent regions of the skeleton in patients with suspected Paget’s disease. […] If the diagnosis is confirmed, we suggest that a radionucleotide bone scan be done to determine the extent of the disease. […] After diagnosis of Paget’s disease, we recommend measurement of serum total alkaline phosphatase or, when warranted, a more specific marker of bone formation or bone resorption to assess the response to treatment or evolution of the disease in untreated patients. […] Serial radionuclide bone scans may determine the response to treatment if the markers are normal.

• #46 Paget Disease of Bone for Primary Care | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p224.html

  All patients who are thought to have Paget disease of bone should have plain radiography of the suspected sites. […] In asymptomatic patients with elevated levels of total serum alkaline phosphatase and no underlying liver or biliary tract disease, plain radiographs of the abdomen, skull and facial bones, and tibia should be obtained. […] Patients diagnosed with Paget disease of bone should have a radionuclide bone scan to fully assess the extent of the disease. […] Measurement of alkaline phosphatase in conjunction with liver function testing is used as a first-line biochemical evaluation. In suspected cases of Paget disease of bone in which total serum alkaline phosphatase levels are normal or hepatic disease exists, measurement of specific bone markers such as procollagen type I N-terminal propeptide, bone-specific alkaline phosphatase, or urinary cross-linked N-terminal telopeptide of type I collagen is indicated.

• #47 Paget Disease of Bone for Primary Care | AAFP

  https://www.aafp.org/pubs/afp/issues/2020/0815/p224.html

  All patients who are thought to have Paget disease of bone should have plain radiography of the suspected sites. […] In asymptomatic patients with elevated levels of total serum alkaline phosphatase and no underlying liver or biliary tract disease, plain radiographs of the abdomen, skull and facial bones, and tibia should be obtained. […] Patients diagnosed with Paget disease of bone should have a radionuclide bone scan to fully assess the extent of the disease. […] Measurement of alkaline phosphatase in conjunction with liver function testing is used as a first-line biochemical evaluation. In suspected cases of Paget disease of bone in which total serum alkaline phosphatase levels are normal or hepatic disease exists, measurement of specific bone markers such as procollagen type I N-terminal propeptide, bone-specific alkaline phosphatase, or urinary cross-linked N-terminal telopeptide of type I collagen is indicated.

• #48 Paget Disease: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/334607-overview

  Paget disease is a localized disorder of bone remodeling that typically begins with excessive bone resorption followed by an increase in bone formation. […] Measurement of serum alkaline phosphatase in some cases, bone-specific alkaline phosphatase (BSAP) along with several urinary markers, can be useful in the diagnosis of Paget disease. Plain radiographs and bone scanning should be performed upon initial diagnosis. […] The degree of elevation of these biomarkers helps identify the extent and severity of bone turnover. […] Markers of bone turnover that are useful to monitor in persons with Paget disease include the following: Bone-specific alkaline phosphatase (marker of bone formation), Deoxypyridinoline (marker of bone resorption), N -Telopeptide of type I collagen (marker of bone resorption), Alpha-alpha type I C-telopeptide fragments. […] Upon successful treatment of Paget disease, the level of these bone markers is expected to decrease.

• #49 Paget Disease Workup: Approach Considerations, Alkaline Phosphatase, Urinary Markers

  https://emedicine.medscape.com/article/334607-workup

  Plain radiographs are less sensitive than bone scan scintigraphy in the diagnosis of Paget disease. […] Computed tomography (CT) scanning and magnetic resonance imaging (MRI) are not needed for the diagnosis of Paget disease of bone. However, both are useful in the evaluation of complications of Paget disease, such as neoplastic degeneration, articular abnormalities, and spinal involvement with neurologic compromise. […] Bone scanning is the most sensitive test for evaluating the extent of lesions in the whole skeleton affected by Paget disease. Bone scintigraphic abnormalities are observed earlier than radiographic changes during the active stage of Paget disease. […] Articular abnormalities require CT scanning or MRI to delineate the extent of involvement. CT scanning and MRI are useful to diagnose and evaluate neurologic complications, such as basilar invagination, spinal cord compression, or hydrocephalus. […] The major histologic feature of Paget disease is abnormal bony architecture. The 3 distinct phases in Paget disease (osteolytic, mixed, and osteosclerotic) may exist separately or in the same bone at one time.

• #50 Paget Disease of Bone – Musculoskeletal and Connective Tissue Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/musculoskeletal-and-connective-tissue-disorders/paget-disease-of-bone/paget-disease-of-bone

  Once a diagnosis of Paget’s disease of bone is made on x-ray, a radionuclide bone scan using technetium-labeled phosphonates should be done to determine the extent of bone involvement. […] A biopsy is also indicated for patients with known pagetic bone involvement who have worsening symptoms at the affected site to evaluate for osteosarcoma or other tumors (eg, giant cell tumor of bone).

• #51 SciELO Brasil – Diagnosis and treatment of Paget’s disease of bone: a mini-review Diagnosis and treatment of Paget’s disease of bone: a mini-review

  https://www.scielo.br/j/abem/a/g9kNBjYfcqFynGtTdtTGsxg/

  The laboratory hallmark of PDB is increased serum ALP levels, mainly due to the extensive experience with the use of this easily obtainable bone formation marker. […] Plain radiographies of affected bones and bone scintigraphy are most frequently used in diagnosing PDB. […] PDB should be differentiated from rarer disorders, such as fibrous dysplasia of bone, which normally affects individuals under 40 years of age, or hyperostosis frontalis interna, a benign affection of the skull. […] Antiresorptive therapy is the mainstay of PDB medical treatment, and its main objective is to achieve symptom relief, namely bone pain. […] Bisphosphonates are potent antiresorptive agents with high-affinity for bone and, thus, very effective in reducing metabolic activity and achieving symptomatic control in PDB.

• #52 Paget’s disease of bone: when and why to refer to specialist care | British Journal of General Practice

  https://bjgp.org/content/70/700/561

  Pagets disease of bone (PDB) is characterised by increased but disorganised bone remodelling, leading to various complications including pain, deformity, and fracture. […] This article provides guidance to primary care teams on the diagnosis and management of patients with PDB based on a recent clinical guideline. Patients with an isolated elevation in ALP should be considered for radionuclide bone scan imaging as this is the most sensitive way of detecting PDB. […] If bone scans are not readily available, then plain X-rays of the abdomen (including the ribs and femoral heads), both tibias, the skull, and facial bones may be considered since X-rays of these sites have been found to detect PDB in 90% of patients. […] The key investigation is ALP, which gives an indication if PDB is metabolically active, but liver function tests, calcium, phosphate, vitamin D, and creatinine are helpful in the differential diagnosis from other conditions such as renal osteodystrophy, primary hyperparathyroidism, and osteomalacia.

• #53 Sporadic Paget’s disease of the bone. Case series and literature review | Revista Colombiana de Reumatología (English Edition)

  https://www.elsevier.es/en-revista-revista-colombiana-reumatologia-english-edition–474-articulo-sporadic-paget-s-disease-bone-case-S2444440520300455

  Based on the radiographic findings of the disease, it may be classified as active or inactive. […] From the serological point of view, the incidental finding of elevated total alkaline phosphatase (TAP), in relationship to elevated levels of gamma glutamyl transpeptidase (GGT), indicate hepatobiliary pathology. […] TAP is elevated in 85-95% of the patients with untreated PD, it is correlated with the disease activity and therefore may be used as a follow-up biomarker. […] Very rarely, a bone biopsy is required to make the diagnosis, although it may be helpful to differentiate from osteoblastic metastasis or osteosarcomas if the clinic is suggestive.

• #54 Paget’s Disease of Bone: Symptoms, Diagnosis, Treatment

  https://www.webmd.com/osteoporosis/pagets-disease-bone

  Paget’s can be hard to diagnose. It can be confused with arthritis, osteoporosis, spinal stenosis, or other changes that come with age. Some people only find out they have it because of an X-ray or blood test taken for a different reason. […] The doctor may ask you to take blood tests to look for an enzyme known as ALP (alkaline phosphatase) in your blood. People who have Paget’s often have more of this enzyme than normal, which reflects the bone turnover that is common in Paget’s. […] To see if you have Paget’s disease of bone, your doctor will examine you and ask questions about your family history. They’ll want pictures taken of your bones, because a bone with Paget’s will look larger and thicker than usual. It can look like it didn’t grow right. This will be done with an elevated Alk Phos on a routine blood test, xrays that are suspicious for Pagets, or one of the following:

• #55 Treatment of Paget’s Disease of Bone

  https://www.uspharmacist.com/article/treatment-of-pagets-disease-of-bone

  Since Pagets disease often is asymptomatic, it frequently is discovered accidentally because of an elevated serum alkaline phosphatase (ALK-P) of unknown origin or upon radiography performed to evaluate abdominal symptoms or bone, pelvic, or hip pain. Ninety-five percent of untreated patients with Pagets disease have an elevated serum ALK-P level; however, a normal concentration does not exclude the diagnosis. Serum ALK-P may be normal in patients with monostotic or metabolically inactive disease. Serum ALK-P elevations are reflective of an increased number of osteoblasts in the lesions. The level is indicative of both disease extent and disease activity. Serum total ALK-P levels are appropriate for following the disease, but the bone-specific isoform should be used in patients with coexisting hepatobiliary disease.

• #56 Paget’s disease of bone: Report of 11 cases

  https://www.wjgnet.com/2307-8960/full/v9/i14/3478.htm

  PDB is prone to misdiagnosis because of its various nonspecific clinical manifestations and rarity in China. […] The clinical manifestations of PDB are nonspecific, and bone pain, the most common symptom, is constant and tends to worsen at night and upon weight bearing. […] The characteristic features on radionuclide bone scintigraphy are osteolysis during the initial stage, followed by osteogenesis and osteosclerosis. […] The treatment for PDB is aimed at suppressing osteoclast activity and reducing bone turnover from a pathophysiological standpoint. […] Bisphosphonates suppress increased bone resorption and decrease serum ALP and are an ideal choice for treating PDB.

• #57 Paget’s Disease: Causes, Symptoms, and Treatment | Doctor

  https://patient.info/doctor/pagets-disease-of-bone-pro

  Most cases of PDB are diagnosed incidentally on X-rays or as an isolated elevation of serum alkaline phosphatase. Symptomatic patients present with bone pain, deformity, fractures, arthritis and features of compression neuropathy. […] Serum total alkaline phosphatase is recommended as a first-line biochemical screening test in combination with liver function tests in screening for the presence of metabolically active Paget’s disease of bone. […] Radionuclide bone scans, in addition to targeted X-rays, are recommended as a means of fully and accurately defining the extent of metabolically active disease. […] Bone biopsy may be needed if malignant change is suspected. […] The objectives of treatment are control of pain and to reduce or prevent disease progression and complications. […] Referral to secondary care is advisable in a patient thought to have pain or deformity so that further assessment can be performed and treatment offered if appropriate. […] Serial monitoring of alkaline phosphatase is used to monitor the effects of treatment and disease activity.

• #58 Paget’s disease of bone: A clinical update

  https://www1.racgp.org.au/ajgp/2021/january-february/pagets-disease-of-bone

  Initial biochemical evaluation should be done using serum total ALP, which is elevated in untreated PDB and can be used to track the response to treatment. […] The diagnosis of PDB is confirmed by typical radiological findings. […] Plain radiography is recommended for diagnosis of PDB as the pagetic changes are easily recognisable. […] Bone scintigraphy (Tc-99m bone scan) is more sensitive than plain radiology for identifying areas of increased osteoblastic activity and can be used to assess the distribution of asymptomatic disease. […] Treatment response is best assessed by measuring serum total ALP 36 months after treatment and then annually once levels are normalised. […] If symptoms recur and serum ALP rises above the normal range, retreatment with zoledronic acid should be considered.

• #59 Paget’s Disease of Bone | Endocrine Society

  https://www.endocrine.org/clinical-practice-guidelines/pagets-disease-of-bone

  The aim of this guideline was to formulate practice guidelines for the diagnosis and treatment of Paget’s disease of the bone. […] We recommend that plain radiographs be obtained of the pertinent regions of the skeleton in patients with suspected Paget’s disease. […] If the diagnosis is confirmed, we suggest that a radionucleotide bone scan be done to determine the extent of the disease. […] After diagnosis of Paget’s disease, we recommend measurement of serum total alkaline phosphatase or, when warranted, a more specific marker of bone formation or bone resorption to assess the response to treatment or evolution of the disease in untreated patients. […] Serial radionuclide bone scans may determine the response to treatment if the markers are normal.

• #60 Paget’s Disease: Causes, Symptoms, and Treatment | Doctor

  https://patient.info/doctor/pagets-disease-of-bone-pro

  Most cases of PDB are diagnosed incidentally on X-rays or as an isolated elevation of serum alkaline phosphatase. Symptomatic patients present with bone pain, deformity, fractures, arthritis and features of compression neuropathy. […] Serum total alkaline phosphatase is recommended as a first-line biochemical screening test in combination with liver function tests in screening for the presence of metabolically active Paget’s disease of bone. […] Radionuclide bone scans, in addition to targeted X-rays, are recommended as a means of fully and accurately defining the extent of metabolically active disease. […] Bone biopsy may be needed if malignant change is suspected. […] The objectives of treatment are control of pain and to reduce or prevent disease progression and complications. […] Referral to secondary care is advisable in a patient thought to have pain or deformity so that further assessment can be performed and treatment offered if appropriate. […] Serial monitoring of alkaline phosphatase is used to monitor the effects of treatment and disease activity.

• #61 Paget’s disease of bone: when and why to refer to specialist care | British Journal of General Practice

  https://bjgp.org/content/70/700/561

  Referral to secondary care is advisable in a patient thought to have pain or deformity due to PDB so that further assessment can be performed and treatment offered if appropriate. […] Bisphosphonates are the treatment of first choice and are indicated in patients with pain localised to an affected site with evidence of increased metabolic activity.

• #62 Paget’s disease of bone: who to treat and why it matters | Endocrinology Today

  https://endocrinology.medicinetoday.com.au/et/2018/february/feature-article/pagets-disease-bone-who-treat-and-why-it-matters

  Although the prevalence of Pagets disease is declining, it remains an important clinical entity. In people with symptomatic Pagets disease who are left untreated, there is potential for significant disease-specific morbidity. There are highly successful treatments available for this benign bone disorder, with bisphosphonates the mainstay of treatment. […] The diagnosis of Pagets disease is usually straightforward and in most cases is made after detecting an isolated elevation in serum alkaline phosphatase (ALP) levels or finding typical Pagetic changes on a radiograph. The typical features include focal osteolysis with coarsening of the trabecular pattern, bone expansion, and cortical bone thickening. […] Following a diagnosis of Pagets disease, specialist referral of the patient to an endocrinologist is generally recommended if the serum ALP level is elevated or if bone pain is present.

• #63 Paget’s Disease of the Bone: Symptoms, Causes & Treatment

  https://my.clevelandclinic.org/health/diseases/21929-pagets-disease-of-the-bone

  With X-ray images, healthcare providers can see if you have bones affected by Pagets disease. The affected bones look different in the images than unaffected bones. […] Providers sometimes use bone scans to see which bone(s) are affected by Pagets disease and how mild or severe it is. […] There’s currently no cure for Pagets disease of the bone, but its treatable. The sooner Pagets disease can be diagnosed and treated, the less likely that youll experience complications from the disease. […] If you have an elevated alkaline phosphatase blood level, you need an evaluation from a specialist to determine if its coming from your bones. If you have an X-ray thats suggestive of Pagets disease, you should have an evaluation.

• #64 Paget’s Disease of Bone: Symptoms, Diagnosis, Treatment & Complications

  https://resources.healthgrades.com/right-care/endocrinology-and-metabolism/pagets-disease-of-bone

  Paget’s disease of bone is a chronic bone disease. […] Paget’s disease of bone is uncommon. It only affects about 1% of Americans, typically older adults of northern European descent. […] Most people who have Paget’s disease of bone don’t realize it because they have no symptoms. They may get a Paget’s disease of bone diagnosis when they have an X-ray or other imaging exam for another reason. […] Treatment may or may not be necessary if you don’t have symptoms. Your doctor can order a blood test to see if the disease is active or not. The test measures the level of an enzyme, alkaline phosphatase, in your blood. If the level is high, your doctor may recommend treating it, even if you don’t have symptoms. […] Seeing your doctor on a regular basis can help identify signs and symptoms of bone disease early. If you notice new bone or joint symptoms, contact your doctor. Early diagnosis and treatment can help prevent complications and relieve symptoms if you have them.

• #65 SciELO Brasil – Diagnosis and treatment of Paget’s disease of bone: a mini-review Diagnosis and treatment of Paget’s disease of bone: a mini-review

  https://www.scielo.br/j/abem/a/g9kNBjYfcqFynGtTdtTGsxg/

  The laboratory hallmark of PDB is increased serum ALP levels, mainly due to the extensive experience with the use of this easily obtainable bone formation marker. […] Plain radiographies of affected bones and bone scintigraphy are most frequently used in diagnosing PDB. […] PDB should be differentiated from rarer disorders, such as fibrous dysplasia of bone, which normally affects individuals under 40 years of age, or hyperostosis frontalis interna, a benign affection of the skull. […] Antiresorptive therapy is the mainstay of PDB medical treatment, and its main objective is to achieve symptom relief, namely bone pain. […] Bisphosphonates are potent antiresorptive agents with high-affinity for bone and, thus, very effective in reducing metabolic activity and achieving symptomatic control in PDB.

• #66 Paget’s disease of bone: Report of 11 cases

  https://www.wjgnet.com/2307-8960/full/v9/i14/3478.htm

  PDB is prone to misdiagnosis because of its various nonspecific clinical manifestations and rarity in China. […] The clinical manifestations of PDB are nonspecific, and bone pain, the most common symptom, is constant and tends to worsen at night and upon weight bearing. […] The characteristic features on radionuclide bone scintigraphy are osteolysis during the initial stage, followed by osteogenesis and osteosclerosis. […] The treatment for PDB is aimed at suppressing osteoclast activity and reducing bone turnover from a pathophysiological standpoint. […] Bisphosphonates suppress increased bone resorption and decrease serum ALP and are an ideal choice for treating PDB.

• #67 Frequently Asked Questions – Bone Health & Osteoporosis Foundation

  https://www.bonehealthandosteoporosis.org/pagets/pagets-frequently-asked-questions/

  The goal of treatment is to normalize Paget’s disease activity for a prolonged period of time. In general, the therapies of choice are the three more potent bisphosphonates: Actonel™, Fosamax® and Aredia®. Didronel®, Skelid® or Miacalcin® may be appropriate therapies for selected patients. […] There are generally three major complications of Paget’s disease for which surgery may be recommended. The first is fractures in pagetic bone. Surgical repair of fractures in bones with Paget’s disease may help the fractures heal in better position. The second complication occurs when the patient develops severe degenerative arthritis. If medication and physical therapy are no longer helpful, and if disability is severe, joint replacement of the hips and knees may be considered as an option. The third situation involves bone deformity, especially of the tibia. The surgical cutting and realignment of a pagetic bone (osteotomy) may help painful weight-bearing joints, especially the knees.

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