Paget’s disease of bone: an update on epidemiology, pathogenesis and pharmacotherapy

Gennari, Luigi; Rendina, Domenico; Picchioni, Tommaso; Bianciardi, Simone; Materozzi, Maria; Nuti, Ranuccio; Merlotti, Daniela

doi:10.1080/21678707.2018.1500691

Cited by 13 publications

(11 citation statements)

References 127 publications

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“…They are effective in reducing bone turnover and bone pain, and they promote the healing of osteolytic lesions. [ 24 ] However, their efficacy to prevent complications of Paget's disease of bone is still debated. Bisphosphonates have a long residual half-life.…”

Section: Discussionmentioning

confidence: 99%

Paget's disease of bone in Tunisia: A study of 69 patients

Maatallah¹,

Rahmouni²,

Miladi

et al. 2020

Indian J Endocr Metab

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Context: Paget's disease of bone is a common bone disease with a striking variation in its incidence and characteristics in different parts of the world. It is uncommonly reported in African patients. Aims: Given the lack of studies describing the characteristics of patients with Paget's disease of bone in North Africa, we aimed to describe demographic, clinical, biochemical, and imaging characteristics, as well as treatment outcomes of Tunisian patients with Paget's disease of bone. Subjects and Methods: This bicentric and retrospective study included patients with Paget's disease of bone. Clinical, laboratory, radiological profile, and response to treatment were analyzed. Results: Sixty-nine patients were identified. The mean age was 64.9 ± 11.6 years and 52.2% were women. One patient reported a positive family history. Seven patients were asymptomatic. Bone pain was the most common presenting symptom. Eight patients had a history of malignancy. In three patients, Paget's disease of bone was diagnosed as part of a metastatic workup. Monostotic disease was found in half of the cases. The most commonly involved sites were pelvis (43.5%), femur (21.7%), and spine (21.7%). The mean serum alkaline phosphatase level at presentation was 591 U/L (68–8380). Two patients received salmon calcitonin (2.8%) and 47 patients (68.1%) received bisphosphonates. After a mean follow-up of 55 months (2–240 months), bone pain improved in 43.1% of patients and the serum alkaline phosphate levels had normalized in 22 of them (43.1%). During follow-up, there was no malignant transformation. Conclusions: In this series of Tunisian patients, Paget's disease of bone had a female predominance and was usually monostotic. The clinical and radiological presentations were similar to the European series.

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Section: Discussionmentioning

confidence: 99%

Paget's disease of bone in Tunisia: A study of 69 patients

Maatallah¹,

Rahmouni²,

Miladi

et al. 2020

Indian J Endocr Metab

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“…PDB, also called osteitis deformans or osteodystrophia deformans, is a chronic, non-inflammatory, focal bone remodeling disease with pathologically increased bone turnover rates, i.e. enhanced resorption of bone is associated with accelerated, random new bone formation [12–14,35]. In PDB, the number and size of osteoclasts (bone-absorbing cells) is strongly increased with a higher number of nuclei per cell (up to 100), and their bone absorbing activity is enhanced, creating lytic areas and Howship’s lacunae in the affected bones[14,16].…”

Section: Discussionmentioning

confidence: 99%

“…The cause of PDB is still unknown although most research shows that there is a genetic predisposition for susceptibility to this disorder, especially connected with mutations of gene SQSTM1[13,35]. Epidemiological studies indicate declining prevalence rates of PDB in humans, and this suggests that also environmental triggers are interacting with the genetic predisposition [35]. Environmental factors that have been previously discussed include wood-fired heating, tobacco smoking, consumption of brains, rural life, and especially contact with farm or wildlife animals (Cundy & Bolland 2008 and references therein).…”

Section: Discussionmentioning

confidence: 99%

Permian metabolic bone disease revealed by microCT: Paget’s disease-like pathology in vertebrae of an early amniote

et al. 2019

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Bone remodeling is an essential physiological process in growth and healing. In modern systems deviations from normal bone physiology in the form of pathologies aid in the understanding of normal bone metabolism. Here we use external morphology and X-ray microtomography to diagnose and describe a metabolic bone disease in an amniote from the early Permian. The specimen consists of two fused tail vertebrae of a small varanopid from early Permian (289 million years old) cave deposits near Richards Spur, Oklahoma, USA. Inspection of the outer morphology reveals that the fusion encompasses the vertebral centra, zygopophyses and haemal arches, with the fusion zones distinctly swollen on the left side of the specimen. With visualization of its internal structure by microCT, this specimen is diagnosed as a complex metabolic bone disease. The radiological imaging suggests a pathologically high bone turnover rate, as shown by abnormal bone formation in some areas and increased bone resorption in others. This supports that the varanopid suffered from a metabolic bone disease similar to Paget’s disease of bone as seen in humans today, which is linked to both genetic and viral factors. This finding extends the occurrence of Paget-like disease to the early Permian, and–provided a viral component was present–would also be by far the oldest evidence of viral infection in the fossil record.

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“…It affects the axial skeleton asymmetrically, primarily involving the pelvis (60.3%), lumbar spine (35.1%), femur (32.3%), skull (22.2%) and tibia (15.5%) [ 2 ]. The disease is rare before 40–50 years of age, and the prevalence is about 1% in the population aged > 50 years, reaching 5% after 80 years [ 3 ]. Bone architecture in Paget’s disease is completely subverted by a disorganized bone tissue with single or multiple bone alterations, and monostotic and polyostotic forms have been described [ 4 ].…”

Section: Introductionmentioning

confidence: 99%

Clinical and radiological outcomes of total hip arthroplasty in patients affected by Paget’s disease: a combined registry and single-institution retrospective observational study

et al. 2021

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Background Total hip arthroplasty (THA) in patients with Paget’s disease can be associated with technical difficulties related to deformities and altered mechanical bone properties, and hypervascularity leads to significative intra-operative bleeding. The purpose of this registry and single-institution study was to investigate overall survival and causes of failure of THA in pagetic patients, together with an analysis of the clinical and radiological complications. Material and methods Registry-based survival and complication analysis, type of fixation, intra- and post-operative complications, clinical (pharmacological history, blood transfusions, Harris hip score [HHS]) and radiographic (cup orientation, stem axial alignment, osteolysis around the cup and the stem and heterotopic ossification [HO]) data were reviewed. Results In total, 66 patients (27 males and 39 females, mean age at surgery 71.1 years for males and 74.8 years for female) from the registry study presented a 10-year survival of 89.5%. In the institutional study, involving 26 patients (14 males and 12 females, 69 years average) and 29 THAs, hip function improved significantly. Average cup orientation was 40.5°, while varus stem alignment was 13.8%. In total, 52% of hips had heterotopic ossifications. Peri-acetabular osteolysis was in 13.8% of implants and in 45% of hips was found around the stem. Allogenic and autologous blood transfusion rate were 68.2% and 31.8%, respectively, with an average transfusion of 2 units of blood (range 1–6 units). HHS improved by an average of 34 points, with excellent result in 64.3% of patients. Two implants failed, one due to traumatic ceramic head fracture 64 months after surgery, and one due to mobilization of the cup on the second post-operative day. Conclusion THA surgery in Paget’s patients is a safe procedure, and implant survival is only partly affected by bone remodelling and choice of fixation. The post-operative functional outcome is largely similar to that of other patients. Bleeding-related complications are the main complications; a careful pharmacological strategy should be recommended to decrease the risk of transfusions and of HO development. Level of evidence Level III

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Paget’s disease of bone: an update on epidemiology, pathogenesis and pharmacotherapy

Cited by 13 publications

References 127 publications

Paget's disease of bone in Tunisia: A study of 69 patients

Paget's disease of bone in Tunisia: A study of 69 patients

Permian metabolic bone disease revealed by microCT: Paget’s disease-like pathology in vertebrae of an early amniote

Clinical and radiological outcomes of total hip arthroplasty in patients affected by Paget’s disease: a combined registry and single-institution retrospective observational study

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