Miao XY, Wang XL, Lyu ZH, Ba JM, Pei Y, Dou JT, Gu WJ, Du J, Guo QH, Chen K, Mu YM. Paget’s disease of bone: Report of 11 cases. World J Clin Cases 2021; 9(14): 3478-3486 [PMID: 34002161 DOI: 10.12998/wjcc.v9.i14.3478]
Corresponding Author of This Article
Xian-Ling Wang, MD, PhD, Chief Doctor, Department of Endocrinology, The First Medical Center, Chinese PLA General Hospital, No. 28 Fuxing Road, Beijing 100853, China. wangxianling1972@sohu.com
Research Domain of This Article
Endocrinology & Metabolism
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. May 16, 2021; 9(14): 3478-3486 Published online May 16, 2021. doi: 10.12998/wjcc.v9.i14.3478
Paget’s disease of bone: Report of 11 cases
Xin-Yu Miao, Xian-Ling Wang, Zhao-Hui Lyu, Jian-Ming Ba, Yu Pei, Jing-Tao Dou, Wei-Jun Gu, Jin Du, Qing-Hua Guo, Kang Chen, Yi-Ming Mu
Xin-Yu Miao, Xian-Ling Wang, Zhao-Hui Lyu, Jian-Ming Ba, Yu Pei, Jing-Tao Dou, Wei-Jun Gu, Jin Du, Qing-Hua Guo, Kang Chen, Yi-Ming Mu, Department of Endocrinology, The First Medical Center, Chinese PLA General Hospital, Beijing 100853, China
Xin-Yu Miao, Department of Endocrinology, The Second Medical Center & National Clinical Research Center for Geriatric Diseases, Chinese PLA General Hospital, Beijing 100853, China
Author contributions: Miao XY, Wang XL, and Pei Y prepared and collected the case data; Mu YM, Lyu ZH, Ba JM, Dou JT, Gu WJ, Du J, Guo QH, and Chen K analyzed the diagnosis and treatment of the case, and summarized the experience of diagnosis and treatment; Miao XY and Wang XL reviewed the literature and was responsible for drafting the manuscript; all authors gave final approval for the version to be submitted.
Supported bySpecial Scientific Research Project of Military Healthcare, No. 19BJZ29; Beijing Haidian District Health Development Research and Cultivation Program, No. HP-2021-03-80303.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Xian-Ling Wang, MD, PhD, Chief Doctor, Department of Endocrinology, The First Medical Center, Chinese PLA General Hospital, No. 28 Fuxing Road, Beijing 100853, China. wangxianling1972@sohu.com
Received: December 30, 2020 Peer-review started: December 30, 2020 First decision: January 24, 2021 Revised: February 2, 2021 Accepted: March 3, 2021 Article in press: March 3, 2021 Published online: May 16, 2021 Processing time: 119 Days and 20 Hours
Abstract
BACKGROUND
Paget’s disease of bone (PDB) is a rare metabolic bone disease in China and is characterized by increased bone resorption and disorganized bone formation. The main clinical symptoms of PDB are focal or multiple bone pain and deformity with high disability. The disease has high missed diagnosis and misdiagnosis rates. This report summarizes the clinical manifestations, imaging and pathological features, and treatments of 11 patients with PDB at our hospital from 1993 to 2020 in order to improve the recognition and prognosis of PDB.
CASE SUMMARY
There were eight male and three female patients whose average age was 48.7 ± 11.0 years with a PDB course of 1-16 years. Nine patients had bone pain and bone deformities in different parts of the body, the majority of which involved the long bones. Laboratory examinations revealed elevated serum alkaline phosphatase (ALP) in all patients with an average of 618 ± 460 IU/L (normal range 0-130 IU/L), and serum calcium and phosphorus levels were in the normal range. Imageology showed that osteolysis was usually combined with osteosclerosis and/or bone deformities in single or multiple bones. 99mTc-methylene diphosphonate bone scintigraphy revealed increased radionuclide uptake in the bone lesions. Six patients underwent bone tissue biopsy, and the typical pathological changes were a mosaic structure of the bone trabeculae with irregularly arranged cement lines and multinuclear osteoclasts. Ten of the 11 patients were effectively treated with bisphosphonates.
CONCLUSION
Early diagnosis of the rare disease PDB can be made through elevated ALP levels and typical presentations on bone X-ray and from bone tissue biopsy.
Core Tip: Paget’s disease of bone (PDB) is a rare metabolic bone disease in China that has high missed diagnosis and misdiagnosis rates. We retrospectively summarize the clinical data of 11 patients with a diagnosis of PDB to provide additional clinical evidence for the disease. We found that PDB presented with a predisposition toward the older male population, and the main clinical symptoms were bone pain, deformity, and a high risk of disability. Early identification of PDB can be made through elevated alkaline phosphatase levels and typical presentations on bone X-ray and from bone tissue biopsy. Bisphosphonates are the first choice to improve the symptoms and prognosis.
