Clinical features and diagnostic tools in idiopathic inflammatory myopathies

Tsamis, Konstantinos I.; Boutsoras, Constantinos; Kaltsonoudis, Evripidis; Drosos, Alexandros A.; Nikas, Ilias P.; Simos, Yannis V.; Sarmas, Ioannis

doi:10.1080/10408363.2021.2000584

Cited by 7 publications

(10 citation statements)

References 185 publications

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“…Besides the muscle symptoms, extra-muscular manifestations, such as skin rash, arthritis, and interstitial lung disease (ILD), are common, which emphasizes the systemic inflammatory nature of these disorders (1). Along with the discovery of myositis-specific antibodies, IIM is categorized into not only two classical subgroups such as dermatomyositis (DM) and polymyositis (PM), but also two new subgroups with distinct clinical manifestations and muscle histopathological features, which are anti-synthetase syndrome and immune-mediated necrotizing myopathy (1)(2)(3). According to the 239th European NeuroMuscular Center classification criteria for DM, patients with anti-synthetase autoantibodies will be classified as having the anti-synthetase syndrome but not DM (4).…”

Section: Introductionmentioning

confidence: 99%

A Matrix Prediction Model for the 6-Month Mortality Risk in Patients With Anti-Melanoma Differentiation-Associated Protein-5-Positive Dermatomyositis

et al. 2022

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ObjectivesThe purpose of this study was to investigate the baseline independent risk factors for predicting 6-month mortality of patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive dermatomyositis (DM) and develop a matrix prediction model formed by these risk factors.MethodsThe hospitalized patients with DM who completed at least 6-month follow-up were recruited as a derivation cohort. The primary exposure was defined as positive anti-MDA5 at the baseline. The primary outcome was all-cause 6-month mortality after enrollment. A matrix prediction model was developed in the derivation cohort, and another published cohort was used for external validation.ResultsIn derivation cohort, 82 patients with DM were enrolled (mean age of onset 50 ± 11 years and 63% women), with 40 (49%) showing positive anti-MDA5. Gottron sign/papules (OR: 5.135, 95%CI: 1.489–17.708), arthritis (OR: 5.184, 95%CI: 1.455–18.467), interstitial lung disease (OR: 7.034, 95%CI: 1.157–42.785), and higher level of C4 (OR: 1.010, 95%CI: 1.002–1.017) were the independent associators with positive anti-MDA5 in patients with DM. Patients with anti-MDA5-positive DM had significant higher 6-month all-cause mortality than those with anti-MDA5-negative (30 vs. 0%). Among the patients with anti-MDA5-positive DM, compared to the survivors, non-survivors had significantly advanced age of onset (59 ± 6 years vs. 46 ± 9 years), higher rates of fever (75 vs. 18%), positive carcinoma embryonic antigen (CEA, 75 vs. 14%), higher level of ferritin (median 2,858 ug/L vs. 619 ug/L, all p < 0.05). A stepwise multivariate Cox regression showed that ferritin ≥1,250 μg/L (HR: 10.4, 95%CI: 1.8–59.9), fever (HR: 11.2, 95%CI: 2.5–49.9), and positive CEA (HR: 5.2, 95%CI: 1.0–25.7) were the independent risk factors of 6-month mortality. A matrix prediction model was built to stratify patients with anti-MDA5-positive DM into different subgroups with various probabilities of 6-month mortality risk. In an external validation cohort, the observed 6-month all-cause mortality was 78% in high-risk group, 43% in moderate-risk group, and 25% in low-risk group, which shows good accuracy of the model.ConclusionBaseline characteristics such as fever, ferritin ≥1,250 μg/L, and positive CEA are the independent risk factors for 6-month all-cause mortality in patients with anti-MDA5-positive DM. A novel matrix prediction model composed of these three clinical indicators is first proposed to provide a chance for the exploration of individual treatment strategies in anti-MDA5-positive DM subgroups with various probabilities of mortality risk.

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Section: Introductionmentioning

confidence: 99%

A Matrix Prediction Model for the 6-Month Mortality Risk in Patients With Anti-Melanoma Differentiation-Associated Protein-5-Positive Dermatomyositis

et al. 2022

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“…However, no correlations between lean mass assessed by DEXA and physical or mental SF‐36 could be observed in the present study. It is possible, however, that correlations might be expected with more advanced stages of the disease, as muscle quality, 1,2 and muscle function are strongly negatively affected by disease progression over time 5 …”

Section: Discussionmentioning

confidence: 99%

“…1,2 The cornerstone treatment for myositis is immunosuppressive therapy except for inclusion body myositis, a treatment-resistant subgroup of myositis. 1,2 However, QoL appears to remain negatively affected even in patients in remission. [3][4][5] QoL is a complex measure, and it is unclear what factors might affect QoL in myositis.…”

Section: Introductionmentioning

confidence: 99%

Quality of life in patients with myositis is associated with functional capacity, body composition, and disease activity—Baseline data from a randomized controlled trial

Jensen,

Aagaard,

Suetta

et al. 2024

Int J of Rheum Dis

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ObjectiveTo investigate the potential associations between functional capacity, muscle strength, body composition, and disease‐related measures and quality of life in patients with myositis.MethodsBaseline measures of functional capacity (functional index 3 (FI3), 2‐minute walk test (2MWT), timed up and go (TUG) and 30‐s sit‐to‐stand (30‐STS)), muscle strength (incl. leg and handgrip strength), maximal leg extensor power, body composition (appendicular lean mass, fat percentage/mass) and disease‐related measures (disease activity & damage core sets) were examined for their associations with quality of life (physical‐ and mental component summary scores, Short Form 36 questionnaire (SF‐36)) by means of Spearman's correlation analysis.ResultsA total of 32 patients with myositis were included. Positive correlations between SF‐36 physical component summary score (PCS) and FI3, 30‐STS, TUG, 2MWT, leg extensor power, leg strength, bench press strength, and handgrip strength were observed. In contrast, fat percentage and fat mass correlated negatively with PCS. In disease‐related measures, Extramuscular global assessment, health assessment questionnaire, physician global damage, and patient global damage scores were negatively associated with SF‐36 PCS. No correlations to the mental component summary score of SF‐36 were observed.ConclusionAll measures of functional capacity were positively related to the SF‐36 physical component summary score, indicating higher functional capacity positively affects quality of life in patients with myositis. Health assessment questionnaire and patient global damage scores demonstrated the strongest correlations with SF‐36 physical component summary scores, further supporting these patient‐reported outcomes as viable monitoring tools in patients with myositis.

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“…Idiopathic inflammatory myopathies, also termed as myositis, constitute a heterogenous group of autoimmune conditions affecting the skeletal muscles, skin, and internal organs [ 21 ]. IIM can be classified into several subgroups: dermatomyositis (DM, including clinically amyopathic dermatomyositis (CADM)), polymyositis (PM), anti-synthetase syndrome (ASS), inclusion body myositis (IBM), immune-mediated necrotizing myopathy (IMNM), and overlap myositis (OM) [ 22 , 23 , 24 ]; however, the major complaints across different subtypes are muscle weakness and fatigue [ 25 , 26 ]. Despite previous concerns that exercise would exacerbate inflammation in these patients, compelling evidence has proved the opposite: exercise or physiotherapy is an effective non-pharmacological intervention to maintain and improve muscle strength and function in IIM patients [ 27 , 28 , 29 , 30 ], including our recent study on IIM patients after a 24-week exercise intervention [ 31 ].…”

Section: Introductionmentioning

confidence: 99%

Hsp90 as a Myokine: Its Association with Systemic Inflammation after Exercise Interventions in Patients with Myositis and Healthy Subjects

Švec

Štorkánová

Špiritović

et al. 2022

IJMS

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Compelling evidence supports the health benefits of physical exercise on the immune system, possibly through the molecules secreted by the skeletal muscles known as myokines. Herein, we assessed the impact of exercise interventions on plasma Heat shock protein 90 (Hsp90) levels in 27 patients with idiopathic inflammatory myopathies (IIM) compared with 23 IIM patients treated with standard-of-care immunosuppressive therapy only, and in 18 healthy subjects undergoing strenuous eccentric exercise, and their associations with the traditional serum markers of muscle damage and inflammation. In contrast to IIM patients treated with pharmacotherapy only, in whom we demonstrated a significant decrease in Hsp90 over 24 weeks, the 24-week exercise program resulted in a stabilization of Hsp90 levels. These changes in Hsp90 levels were associated with changes in several inflammatory cytokines/chemokines involved in the pathogenesis of IIM or muscle regeneration in general. Strenuous eccentric exercise in healthy volunteers induced a brief increase in Hsp90 levels with a subsequent return to baseline levels at 14 days after the exercise, with less pronounced correlations to systemic inflammation. In this study, we identified Hsp90 as a potential myokine and mediator for exercise-induced immune response and as a potential biomarker predicting improvement after physiotherapy in muscle endurance in IIM.

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Clinical features and diagnostic tools in idiopathic inflammatory myopathies

Cited by 7 publications

References 185 publications

A Matrix Prediction Model for the 6-Month Mortality Risk in Patients With Anti-Melanoma Differentiation-Associated Protein-5-Positive Dermatomyositis

A Matrix Prediction Model for the 6-Month Mortality Risk in Patients With Anti-Melanoma Differentiation-Associated Protein-5-Positive Dermatomyositis

Quality of life in patients with myositis is associated with functional capacity, body composition, and disease activity—Baseline data from a randomized controlled trial

Hsp90 as a Myokine: Its Association with Systemic Inflammation after Exercise Interventions in Patients with Myositis and Healthy Subjects

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