Abstract
Although Paget’s disease of bone (PDB) is the second most common metabolic bone disease, there is only limited information about the microarchitecture of affected bones. Therefore, the aim of this study was to determine cortical and trabecular bone properties in clinically relevant locations by microcomputed tomography (µCT). Ten femora and ten tibiae affected by Paget’s disease taken from the Natural History Museum Vienna were compared to 13 femora and 10 tibiae of non-affected body donors. Digitization of the cortical and trabecular bone microarchitecture was performed with an X-ray-based µCT scanner. Additionally, semi-quantitative gradings of trabecular and cortical architectural parameters of the femora and the tibiae were generated. Microcomputed tomography images showed changes in the thickness of cortices, cortical porosity, and trabecularization of cortical structures. Moreover, severe disorganization of trabecular structures, trabecular defects, and thickening of (remaining) trabeculae were detected. Numerical cortical analyses showed lower total bone volume (BV) and lower BV in the outer region (66–100%) (− 36%, p = 0.004, and − 50%, p < 0.001, respectively), lower total volume (TV) in the outer region (66–100%) (− 42%, p < 0.001), lower total bone volume fraction (BV/TV) and BV/TV in the outer region (66–100%) (− 23%, and − 12%, p < 0.001, respectively), higher BV and TV in the middle region (33–66%) and higher BV/TV in the inner region (0–33%) (123%, p = 0.011, 147%, p = 0.010, and 33%, p = 0.025, respectively) in Pagetic compared to non-affected bones. Trabecular analyses showed higher BV/TV (96%, p = 0.008) and Tb.Th (43%, p = 0.004) in Pagetic compared to non-affected bones. There is a major and consistent structural alteration of PDB at cortical and trabecular sites in weight-bearing long bones. Our findings are relevant for the differential diagnosis of PDB and for the pathogenesis of associated complications, since the disorder produces abnormalities in the structure that might lead to bone fragility.
Highlights
Paget’s disease of bone (PDB), known as Osteitis deformans, was originally described by Sir James Paget in 1877
Analysis of the femoral cortical parameters showed lower total bone volume (BV) and lower bone volume in the outer region (66–100%) (− 36%, p = 0.004, and − 50%, p < 0.001, respectively), lower total volume (TV) in the outer region (66–100%) (− 42%, p < 0.001), and lower total BV/TV and BV/TV in the outer region (66–100%) (− 23%, and − 12%, p < 0.001, respectively) in Pagetic compared to non-affected bones
Consistent with the imaging findings, numerical analyses in this study have shown that BV/TV was lower in Pagetic femoral cortical midshafts than in the non-affected bones
Summary
Paget’s disease of bone (PDB), known as Osteitis deformans, was originally described by Sir James Paget in 1877. The disease is a chronic bone abnormality, which may affect a single, several, or many bones but never involves the entire skeleton. The axial skeleton is frequently involved, and the bones most commonly affected include the pelvis (70%), femur (55%), lumbar spine (53%), skull (42%), and tibia (32%) [2, 3]. Pagetic bone lesions can occur at any site of the skeleton [4]. The diagnosis of PDB is rare before the age of 50. The disease affects both sexes [5], but males have been reported to show a higher frequency. Most patients are asymptomatic [8], while others develop complications such as bone pain, osteoarthritis, fracture, deformity, deafness, and nerve compression syndromes [4]
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