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      Bone mineral density and explanatory factors in children and adults with juvenile dermatomyositis at long term follow-up; a cross sectional study


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          Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in children and adolescents. Both the disease and its treatment with glucocorticoids may negatively impact bone formation. In this study we compare BMD in patients (children/adolescence and adults) with long-standing JDM with matched controls; and in patients, explore how general/disease characteristics and bone turnover markers are associated with BMD .


          JDM patients ( n = 59) were examined median 16.8y (range 6.6–27.0y) after disease onset and compared with 59 age/sex-matched controls. Dual-energy X-ray absorptiometry (DXA) was used to measure BMD of the whole body and lumbar spine (spine) in all participants, and of ultra-distal radius, forearm and total hip in participants ≥20y only. Markers of bone turnover were analysed, and associations with outcomes explored.


          Reduced BMD Z-scores (<−1SD) were found in 19 and 29% of patients and 7 and 9% of controls in whole body and spine, respectively ( p-values < 0.05). BMD and BMD Z-scores for whole body and spine were lower in all patients and for < 20y compared with their respective controls. In participants ≥20y, only BMD and BMD Z-score of forearm were lower in the patients versus controls. In patients, BMD Z-scores for whole body and/or spine were found to correlate negatively with prednisolone use at follow-up (yes/no) (age < 20y), inflammatory markers (age ≥ 20y) and levels of interferon gamma-induced protein 10 (IP-10) (both age groups). In all patients, prednisolone use at follow-up (yes/no) and age ≥ 20y were independent correlates of lower BMD Z-scores for whole body and spine, respectively.


          In long-term JDM, children have more impairment of BMD than adults in spine and whole-body. Associations with BMD were found for both prednisolone and inflammatory markers, and a novel association was discovered with the biomarker of JDM activity, IP-10.

          Supplementary Information

          The online version contains supplementary material available at 10.1186/s12969-021-00543-z.

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          Most cited references40

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          Polymyositis and dermatomyositis (first of two parts).

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            Bone loss in inflammatory disorders.

            Chronic inflammatory diseases of almost any cause are associated with bone loss. Bone loss is due to direct effects of inflammation, poor nutrition, reduced lean body mass, immobility and the effects of treatments, especially glucocorticoids. These mechanisms are complex and interrelated but are ultimately mediated through effects on the bone remodelling cycle. Inflammatory disease can increase bone resorption, decrease bone formation but most commonly impacts on both of these processes resulting in an uncoupling of bone formation from resorption in favour of excess resorption. This review will illustrate these interactions between inflammation and bone metabolism and discuss how these are, and might be, manipulated as therapies for inflammation related bone loss.
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              Role of phosphate sensing in bone and mineral metabolism


                Author and article information

                Pediatr Rheumatol Online J
                Pediatr Rheumatol Online J
                Pediatric Rheumatology Online Journal
                BioMed Central (London )
                26 April 2021
                26 April 2021
                : 19
                : 56
                [1 ]GRID grid.5510.1, ISNI 0000 0004 1936 8921, Institute for Experimental Medical Research and KG Jebsen Center for Cardiac Research, , University of Oslo and Oslo University Hospital, ; Ullevål, 0027 Oslo, Norway
                [2 ]GRID grid.510411.0, ISNI 0000 0004 0578 6882, Bjørknes University College, ; Oslo, Norway
                [3 ]GRID grid.55325.34, ISNI 0000 0004 0389 8485, Section of Specialized Endocrinology, Department of Endocrinology, Morbid Obesity and Preventive Medicine, Medical Clinic, , Oslo University Hospital, ; Oslo, Norway
                [4 ]GRID grid.5510.1, ISNI 0000 0004 1936 8921, Institute for Clinical Medicine, Medical Faculty, , University of Oslo, ; Oslo, Norway
                [5 ]GRID grid.55325.34, ISNI 0000 0004 0389 8485, Department of Rheumatology, , Oslo University Hospital, Rikshospitalet, ; Oslo, Norway
                [6 ]GRID grid.55325.34, ISNI 0000 0004 0389 8485, Department of Cardiology, , Oslo University Hospital Ullevål, ; Oslo, Norway
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                © The Author(s) 2021

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                : 13 November 2020
                : 14 April 2021
                Research Article
                Custom metadata
                © The Author(s) 2021

                juvenile dermatomyositis,dxa,bone mineral density,inflammatory markers,ip-10,prednisolone
                juvenile dermatomyositis, dxa, bone mineral density, inflammatory markers, ip-10, prednisolone


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