scholarly journals AB0671 Increased body fat but decreased lean body mass and bone mineral density in patients with idiopathic inflammatory myopathies are associated with disease duration, inflammatory status, skeletal muscle involvement and physical activity

2017 ◽  
Author(s):  
S Oreska ◽  
M Spiritovic ◽  
P Cesak ◽  
O Marecek ◽  
H Storkanova ◽  
...  
Keyword(s):  
Physical Activity ◽  
Bone Mineral Density ◽  
Skeletal Muscle ◽  
Disease Duration ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Mineral Density ◽  
Muscle Involvement ◽  
Inflammatory Status ◽  
Skeletal Muscle Involvement

scholarly journals THU0358 NEGATIVE CHANGES OF BODY COMPOSITION IN MYOSITIS PATIENTS AND THEIR ASSOCIATION WITH DISEASE SPECIFIC CHARACTERISTICS, PHYSICAL ACTIVITY AND NUTRITIONAL STATUS.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 410.3-410
Author(s):  
S. Oreska ◽  
M. Špiritović ◽  
P. Česák ◽  
O. Marecek ◽  
H. Štorkánová ◽  
...  
Keyword(s):  
Physical Activity ◽  
Skeletal Muscle ◽  
Body Composition ◽  
Lipid Metabolism ◽  
Nutritional Status ◽  
Disease Activity ◽  
Inflammatory Cytokines ◽  
Disease Duration ◽  
Serum Levels ◽  
Muscle Involvement

Background:Skeletal muscle, pulmonary and articular involvement in idiopathic inflammatory myopathies (IIM) limit the mobility/self-sufficiency of patients, and can have a negative impact on body composition.Objectives:The aim was to assess body composition and physical activity of IIM patients and healthy controls (HC) and the association with selected inflammatory cytokines/chemokines and laboratory markers of nutrition and lipid metabolism.Methods:54 patients with IIM (45 females; mean age 57.7; disease duration 5.8 years; polymyositis (PM, 22) / dermatomyositis (DM, 25) / necrotizing myopathy (IMNM, 7)) and 54 age-/sex-matched HC (45 females, mean age 57.7) without rheumatic/tumor diseases were included. PM/DM patients fulfilled Bohan/Peter criteria for PM/DM. We assessed body composition (densitometry: iDXA Lunar, bioelectric impedance: BIA2000-M), physical activity (Human Activity Profile, HAP questionnaire), serum levels of 27 cytokines/chemokines (commercial multiplex ELISA kit, Bio-Rad Laboratories) and serum levels of selected parameters of nutrition and lipidogram. Disease activity (MITAX and MYOACT activity score) and muscle involvement (manual muscle testing, MMT-8, and functional index 2, FI2) were evaluated. Data are presented as mean±SD.Results:Compared to HC, patients with IIM had a trend towards significantly increased body fat % (BF%; iDXA: 39.9±7.1 vs. 42.4±7.1 %, p=0.077), but significantly decreased lean body mass (LBM; iDXA: 45.6±8.1 vs. 40.6±7.2 kg, p=0.001; BIA: 52.6±8.8 vs. 48.7±9.0 kg, p=0.023), increased extracellular mass/body cell mass (ECM/BCM) ratio (1.06±0.15 vs. 1.44±0.42, p<0.001), reflecting deteriorated nutritional status and predisposition for physical activity, and significantly lower bone mineral density (BMD: 1.2±0.1 vs. 1.1±0.1 g/cm2, p<0.001). Disease duration negatively correlated with BMD and LBM-BIA. Disease activity (MITAX, MYOACT) positively correlated with LBM (by BIA and DXA), similarly as with basal metabolic rate (BMR), and fat free mass (FFM). CRP was positively associated with BF% (BIA and DXA). Higher BF%-DEXA was associated with worse physical endurance (FI2) and worse ability to perform physical activity (HAP). MMT-8 score negatively correlated with ECM/BCM ratio. Serum levels of several inflammatory cytokines/chemokines (specifically IL-1ra, MCP, IL-10) and markers of nutrition (specifically albumin, C3-, C4-complement, cholinesterase, amylase, insulin and C-peptide, vitamin-D, orosomucoid), and lipid metabolism (specifically triglycerides, high-density lipoprotein, apolipoprotein A and B, atherogenic index of plasma) were significantly associated with alterations of body composition in IIM patients. (p<0.05 for all correlations)Conclusion:Compared to healthy age-/sex-matched individuals we found significant negative changes in body composition of our IIM patients associated with their disease activity and duration, inflammatory status, skeletal muscle involvement, and physical activity. These data could reflect their impaired nutritional status and predispositions for physical exercise, aerobic fitness and performance.Serum levels of certain inflammatory cytokines/chemokines and markers of nutrition and lipid metabolism were associated with alterations of body composition in IIM patients. This might further support the role of systemic inflammation and nutritional status on the negative changes in body composition of IIM patients.Acknowledgments:Supported by AZV NV18-01-00161A, MHCR 023728, SVV 260373 and GAUK 312218Disclosure of Interests:Sabina Oreska: None declared, Maja Špiritović: None declared, Petr Česák: None declared, Ondrej Marecek: None declared, Hana Štorkánová: None declared, Barbora Heřmánková: None declared, Kateřina Kubinova: None declared, Martin Klein: None declared, Lucia Vernerová: None declared, Olga Růžičková: None declared, Karel Pavelka Consultant of: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Speakers bureau: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Ladislav Šenolt: None declared, Heřman Mann: None declared, Jiří Vencovský: None declared, Michal Tomčík: None declared

scholarly journals THU0365 INCREASED HSP90 IN MUSCLE TISSUE AND PLASMA ASSOCIATES WITH DISEASE ACTIVITY AND SKELETAL MUSCLE INVOLVEMENT IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 414.1-414
Author(s):  
H. Štorkánová ◽  
S. Oreska ◽  
M. Špiritović ◽  
B. Heřmánková ◽  
O. Kryštůfková ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Disease Activity ◽  
Inflammatory Cells ◽  
Healthy Controls ◽  
Inflammatory Myopathies ◽  
Muscle Enzymes ◽  
Muscle Involvement ◽  
Hsp90 Expression ◽  
Muscle Biopsies ◽  
Skeletal Muscle Involvement

Background:Heat shock proteins (Hsps) are chaperones playing important roles in skeletal muscle physiology, adaptation to exercise or stress, and activation of inflammatory cellsObjectives:The aim of our study was to assess Hsp90 expression in muscle biopsies and plasma of patients with idiopathic inflammatory myopathies (IIM) and to characterize its association with IIM-related features.Methods:Total of 277 patients with IIM (198 females, 79 males; mean age 54.8; disease duration 4.1 years; DM, 104/PM, 108/CADM, 31/IMNM, 25) and 157 healthy individuals (92 females, 65 males; mean age 47.0) were included in plasma analysis. Muscle biopsy samples (PM, DM, IMNM, myodystrophy, myasthenia gravis) were stained for Hsp90α (Thermo Fisher Scientific, USA) and Hsp90β (Abcam, UK). Plasma Hsp90 was measured by ELISA kit (eBioscience, Vienna, Austria). The cytokines/chemokines were analysed by using Bio-Plex ProTMhuman Cytokine 27-plex Assay (BIO-RAD, California, USA.Data are presented as median(IQR).Results:In muscle biopsies, Hsp90 expression of both subunits (alpha and beta) was higher in IIM than in controls. Increased Hsp90 was detected in perifascicular degenerating and regenerating fibers, inflammatory cells (DM, PM), and necrotic and regenerating fibers (IMNM). Plasma Hsp90 levels were increased in IIM patients compared to healthy controls (55.9 (46.9 – 62.5)vs 9.76(7.5 – 13.8), p<0.0001), and in individual subgroups of IIM vs. healthy controls (DM-22.01(14.1 – 41.2), PM-19.7(14.3 – 42.2), CADM-18.9(11.7 – 29.7), IMNM-19.6(16.3 – 45.5), p<0.0001 for all). Hsp90 was higher in males compared to females (p=0.040) and in patients with ILD (p=0.003), cardiac involvement (p=0.004), dysphagia (p=0.018) and presence of anti-Ro52 (p=0.036). Hsp90 levels in all patients positively correlated with muscle enzymes (Tab.1). Hsp90 was associated with disease activity and skeletal muscle involvement (Tab.1). Out of all clinical parameters listed in above-mentioned univariate analysis, in multiple regression analysis Hsp90 levels in IIM patients were significantly affected by muscle enzymes only (p<0.0001, β=0.345). Furthermore, Hsp90 positively correlated with some crucial cytokines involved in pathogenesis of myositis (Tab. 1).Tab 1Clinical parametersSpearman’s rp – valueLDH; AST; ALT0.554; 0.383; 0.181< 0.0001; < 0.0001; 0.003PtDGA; PhDGA; MITAX; MYOACT0.223; 0.217; 0.175; 0.159< 0.001; < 0.001; 0.004; 0.012Pulmonary disease activity0.2010.001Muscle disease activity0.1460.018MMT8, total score; m. biceps brachii; m. gluteus maximus; m. iliopsoas-0.126; -0.125; -0.159; -0.1430.042; 0.043; 0.011; 0.023MDI – Myositis damage index – severity0.1500.041Current Prednisone equivalent dose0.1830.006Cytokines:IL-1b; IL-2; IL-4; IL-6; IFN-γ0.188; 0.269; 0.190; 0.182; 0.2290.002; < 0.0001; 0.002; 0.003; < 0.0001Conclusion:We demonstrate increased Hsp90 expression in IIM muscle biopsy samples, specifically in inflammatory cells, degenerating, regenerating and/or necrotic fibers. Increased Hsp90 plasma levels in IIM patients are associated with disease activity and damage, and with the involvement of proximal skeletal muscles, heart and lungs.Acknowledgments:Supported by AZV-16-33542A, MHCR 023728 and SVV – 260373.Disclosure of Interests:Hana Štorkánová: None declared, Sabina Oreska: None declared, Maja Špiritović: None declared, Barbora Heřmánková: None declared, Olga Kryštůfková: None declared, Heřman Mann: None declared, Martin Komarc: None declared, Josef Zámečník: None declared, Karel Pavelka Consultant of: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Speakers bureau: Abbvie, MSD, BMS, Egis, Roche, UCB, Medac, Pfizer, Biogen, Jiří Vencovský: None declared, Ladislav Šenolt: None declared, Michal Tomcik: None declared

scholarly journals AB0616 REDUCED BONE MINERAL DENSITY IN PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES: A LONGITUDINAL STUDY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1603.3-1604
Author(s):  
Y. K. Tang ◽  
H. So ◽  
T. L. V. Wong ◽  
H. T. Pang ◽  
V. Lao ◽  
...  
Keyword(s):  
Bone Mineral Density ◽  
Bone Mineral ◽  
Bone Health ◽  
High Dose ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Mineral Density ◽  
High Dose Corticosteroid ◽  
Dose Corticosteroid

Background:Reduced bone mineral density (BMD) leads to fragility fracture which is associated with a significant morbidity and excess mortality [1,2]. Patients with idiopathic inflammatory myopathies (IIM) should be at a heightened risk of reduced BMD as a result of the systemic inflammation, reduced mobility and corticosteroid use [3]. A previous cross-sectional study demonstrated a high prevalence of osteoporosis (23.7%) and osteopenia (47.4%) in a cohort of IIM patients [4]. However, longitudinal data are lacking.Objectives:To assess the BMD of IIM patients longitudinally and to investigate the factors associated with accelerated bone loss.Methods:This is a single centered observational study. Existing adult Chinese patients with IIMs who had serial BMD measurements done were recruited. The diagnosis of IIMs was based on the Bohan and Peter’s criteria with definite or probable cases being included [5]. Patients with clinically amyopathic disease must have the typical Gottron’s papules or heliotrope rash as determined by rheumatologists or dermatologists, and with no symptoms or signs of muscle involvement according to Sontheimer [6]. BMD was measured by dual energy X-ray absorptiometry (DEXA). Clinical variables thought to be associated with bone health were documented.Results:All together 28 patients were studied. The mean age of the patients at disease onset was 46.1 years (S.D. 12.2). There was a female predominance (92.9%). The subgroups of IIMs were: dermatomyositis (39.3%), polymyositis (25.3%), clinically amyopathic dermatomyositis (21.4%) and immune mediated necrotising myopathy (14.3%). Only a minority of the patients smoked (7.1%) and none of them drinks regularly. About one fifth of the patients were underweight. All patients have been exposed to systemic corticosteroid, while 82.1% of them were still on it between the two scans with 32.1% even on high dose (>0.5mg prednisolone/kg/day). Three out of the 28 patients (10.7%) was found to be osteoporotic at baseline and 17 patients (60.7%) were osteopenic. Follow-up DEXAs were performed mostly 5 to 10 years after the initial scan. Despite 8 patients (28.6%) were given active anti-osteoporotic medications, the bone health deteriorated significantly. The mean baseline neck of femor BMD dropped from 0.711 to 0.657 g/cm2 (p=0.042) on follow-up, while the total lumbar BMD from 0.951 to 0.905 g/cm2 (p=0.036). The T-score in 11 patients (39.3) reached osteoporotic range at the second DEXA. Together with the patients with osteopenia, 78.6% of the IMM patients had reduced BMD at the follow-up scan. Actually, 5 patients (17.9%) already had one episode of fragility fracture. The use of high dose corticosteroid in between the 2 scans was found to be associated with a greater degree of mean BMD loss in the hip (-0.171 vs -0.007 g/cm2, p=0.007).Conclusion:Reduced BMD is prevalent in patients with IIM. Follow-up study revealed significant worsening of bone health. High dose corticosteroid use might be especially detrimental. Liberal assessment of BMD and use of anti-osteoporotic drugs in IIM patients are advisable. Prompt use of steroid-sparing agents to minimize steroid exposure may also be helpful.References:[1]Falch J, Aho H, Berglund K, et al. Hip fractures in Nordic Cities: difference in incidence. Ann Chir Gynaecol 1995;84:286-90.[2]Dennison E, Mohammed MA, Cooper C. Epidemiology of osteoporosis. Rheum Dis Clin North Am 2006;32:617-29.[3]Luigi S, Massimo V, Giuseppe G. Epidemiology of osteoporosis in rheumatic disease. Clin Exp Rheumatol 2006;32:631-58.[4]So H, Yip ML, Wong KM. Prevalence and associated factors of reduced bone mineral density in patients with idiopathic inflammatory myopathies. Int J Rheum Dis 2016;19:521-8.[5]Bohan A, Peter JB. Polymyositis and dermatomyositis. N Engl J Med 1975;292:344-7.[6]6. Sontheimer RD. Clinically myopathic dermatomyositis: what can we now tell our patients? Arch Dermatol 2010;146:76-80.Disclosure of Interests:None declared

scholarly journals A review of accelerometer-derived physical activity in the idiopathic inflammatory myopathies

2019 ◽  
Vol 3 (1) ◽  
Author(s):  
Alexander Oldroyd ◽  
Max A. Little ◽  
William Dixon ◽  
Hector Chinoy
Keyword(s):  
Physical Activity ◽  
Disease Activity ◽  
Disease Duration ◽  
Idiopathic Inflammatory Myopathies ◽  
Activity Levels ◽  
Inflammatory Myopathies ◽  
Activity Data ◽  
Activity Assessment ◽  
Physical Activity Levels ◽  
Disease Activity Assessment

Abstract Background The idiopathic inflammatory myopathies (IIMs) are a group of rare conditions characterised by muscle inflammation (myositis). Accurate disease activity assessment is vital in both clinical and research settings, however, current available methods lack ability to quantify associated variation of physical activity, an important consequence of myositis. This study aims to review studies that have collected accelerometer-derived physical activity data in IIM populations, and to investigate if these studies identified associations between physical and myositis disease activity. Methods A narrative review was conducted to identify original articles that have collected accelerometer-derived physical activity data in IIM populations. The following databases were searched from February 2000 until February 2019: Medline via PubMed, Embase via OVID and Scopus. Results Of the 297 publications screened, eight studies describing accelerometer use in 181 IIM cases were identified. Seven out of the eight studies investigated juvenile dermatomyositis (JDM) populations and only one reported on an adult-onset population. Population sizes, disease duration, accelerometer devices used, body placement sites, and study duration varied between each study. Accelerometer-derived physical activity levels were reduced in IIM cohorts, compared to healthy controls, and studies reported improvement of physical activity levels following exercise programme interventions, thus demonstrating efficacy. Higher levels of accelerometer-derived physical activity measurements were associated with shorter JDM disease duration, current glucocorticoid use and lower serum creatine kinase. However, no clear association between muscle strength and accelerometer-derived physical activity measures was identified. Conclusions The use of accelerometer-derived physical activity in IIM research is in its infancy. Whilst knowledge is currently limited to small studies, the opportunities are promising and future research in this area has the potential to improve disease activity assessment for clinical and research applications.

A Case of Immune-Mediated Necrotizing Myopathy With Associated Skeletal Muscle Involvement by Sarcoid Granulomata: A Rare Association

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S69-S69
Author(s):  
Shalla Akbar ◽  
Sandhya Dasaraju ◽  
Osama Elkadi
Keyword(s):  
Skeletal Muscle ◽  
Muscle Weakness ◽  
Inflammatory Myopathies ◽  
Proximal Muscle Weakness ◽  
Proximal Muscle ◽  
Muscle Enzymes ◽  
Muscle Involvement ◽  
Necrotizing Myopathy ◽  
Immune Mediated ◽  
Skeletal Muscle Involvement

Abstract Skeletal muscle involvement by noncaseating granulomata occurs in a variety of conditions, including sarcoidosis, infections, and rarely in association with primary inflammatory myopathies such as inclusion body myositis (IBM) and dermatomyositis (DM). Sarcoid myopathy is typically asymptomatic; however, a picture of acute myositis with proximal muscle weakness has been described. Immune-mediated necrotizing myopathy (IMNM) is a subgroup of inflammatory myopathies typically presenting with proximal muscle weakness and markedly elevated muscle enzymes, mostly occurring in the setting of statin treatment. IMNM is associated with positive autoantibodies, but a subset of cases is antibody negative. Here we describe a case of myopathy occurring in association with sarcoidosis with combined features of granulomatous and necrotizing myopathy. The patient was a 54-year-old African American male with medical history significant for statin use 3 years ago, which was discontinued due to myalgia and elevated muscle enzymes and biopsy-proven sarcoidosis diagnosed on a pulmonary lymph node biopsy. He presented with progressive worsening of bilateral proximal weakness involving the upper and lower extremities. Electromyogram showed features of active myopathy with no evidence of peripheral neuropathy. Myositis panel was negative for the following antibodies: anti-Jo1, Mi-2, anti-Ku, PL-7, PL-12, OJ, EJ, and SRP. However, there was elevation of aldolase, CRP, and CK-MB. Biopsy of thigh and deltoid muscle showed necrotic muscle fibers, myophagocytosis with associated minimal inflammation, and multiple well-formed nonnecrotizing granulomas with multinucleated giant cells. Myopathic features include increased internalized nuclei, round atrophic fibers, and scattered split fibers. Specific features of IBM or DM were not present. Conclusion Myopathies developing or worsening after discontinuation of statin are rare. The association of necrotizing myopathy with sarcoidosis is not well described in the literature. Additional studies are warranted to elucidate this association.

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