Bone mineral density and bone microarchitecture in a cohort of patients with Erdheim-Chester Disease
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(2020) 15:236
RESEARCH
Open Access
Bone mineral density and bone microarchitecture in a cohort of patients with Erdheim-Chester Disease Tianhua He1†, Lijia Cui2†, Na Niu3, Fengdan Wang4, Huilei Miao1, Hao Zhao1, Xuemin Gao1, Chang Liu2, Fan Yu2, Yan Jiang2, Ou Wang2, Mei Li2, Xiaoping Xing2, Daobin Zhou1, Jian Li1, Xinxin Cao1* and Weibo Xia2*
Abstract Background: Erdheim-Chester Disease (ECD) is a rare type of non-Langerhans histiocytosis. Skeletal structures are affected in over 95% ECD patients. Due to the lack of proper imaging assessment tools, the alteration of bone microarchitecture in ECD has not been well studied. High-resolution peripheral quantitative computed tomography (HR-pQCT) is a newly developed assessment of bone mineral density and bone microarchitecture. Methods: We performed a cross-sectional study with 13 patients diagnosed with ECD in Peking Union Medical College Hospital between October 2018 and June 2019. The diagnosis of ECD was based on typical pathological findings in the context of appropriate clinical and radiological manifestations. Bone geometry, volumetric bone mineral density and bone microarchitecture of those ECD patients were assessed using HR-pQCT at the nondominant distal radius and distal tibia. Those HR-pQCT parameters were then compared to an ongoing populationbased database of HR-pQCT for Mainland Chinese. Results: As a result, remarkable heterogeneity of osteosclerosis in the HR-pQCT images was found in ECD patients, ranging from apparent normal structure, scattered thickening of trabecula, to homogenous consolidation. In terms of quantitative measurements, total volumetric BMD (383.50 mg/cm3, 1.352 times of normal mean, p = 0.023) of the tibia differed significantly in ECD patients, due to the increased trabecular volumetric BMD (291 mg/cm3, 2.058 times of normal mean, p = 0.003). The increased trabecular volumetric BMD of tibia was associated with remarkably increased number of trabecula (1.7/mm, 1.455 times of normal mean, p = 0.002) and increased thickness of trabecula (0.37 mm, 1.466 times of normal mean, p = 0.003). These differences could be due to the existence of dense bone interposed in the trabecula. Conclusion: This study is the first to assess the volumetric bone mineral density and bone microstructure with HRpQCT in a cohort of ECD patients and indicated that the application of HR-pQCT may help to reveal the nature of bone lesions in the disease. Keywords: Non-Langerhans histiocytosis, HR-pQCT, Chinese
* Correspondence: [email protected]; [email protected] † Tianhua He and Lijia Cui contributed equally to this work. 1 Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China 2 Department of Endocrinology, Key Laboratory of Endocrinology, Ministry of Health, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China Full list of author information is available at the end of the article © The Author(s). 2020 Open Access This article is licensed und
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