Bone Density, Microarchitecture, and Geometry Assessment in Patients with Pachydermoperiostosis Using
Durairaj Arjunan1, Jayaditya Ghosh1, Sayka Barry2
1Department of Endocrinology, PGIMER, Chandigarh, 160012, India.
Pachydermoperiostosis (PDP) patients exhibit reduced cortical bone density and altered bone structure, indicating a higher fracture risk despite normal bone turnover markers. Ongoing monitoring is crucial for managing this rare genetic disorder.
Area of Science:
- Bone Biology
- Genetic Disorders
- Rheumatology
Background:
- Pachydermoperiostosis (PDP) is a rare genetic disorder characterized by periostosis, digital clubbing, and skin thickening.
- Bone health implications in PDP, particularly concerning bone density and microarchitecture, remain under-investigated despite potential systemic inflammation.
- Understanding these bone changes is crucial for assessing fracture risk in PDP patients.
Purpose of the Study:
- To investigate the impact of Pachydermoperiostosis (PDP) on bone density, microarchitecture, and biomechanical properties.
- To compare bone health parameters in treatment-naïve PDP patients with age and BMI-matched healthy controls.
Main Methods:
- Cross-sectional case-control study involving 8 PDP patients and healthy controls.
- Clinical examination, biochemical parameter assessment (calcium, phosphorus, ALP, 25(OH)D, iPTH), and bone turnover markers (CTX, P1NP).
- Dual-energy X-ray absorptiometry (DXA) and high-resolution peripheral quantitative computed tomography (HR-pQCT) for bone density and microarchitecture analysis, supplemented by Finite Element Analysis (FEA).
Main Results:
- HR-pQCT revealed diminished cortical volumetric bone mineral density (vBMD) and increased cortical porosity in PDP patients.
- Bone geometry showed increased cross-sectional bone area in both cortical and trabecular compartments.
- FEA indicated reduced failure load and stiffness in PDP patients, suggesting compromised bone strength, despite similar biochemical and bone turnover markers compared to controls.
Conclusions:
- Individuals with Pachydermoperiostosis (PDP) present with reduced cortical vBMD and disrupted bone microarchitecture.
- These skeletal alterations may be linked to PGE2-mediated inflammation and bone resorption.
- The findings suggest an increased risk of fractures in PDP patients, necessitating regular monitoring and further research into management strategies.
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