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Scientific Reports May 2021To develop a machine learning (ML) model that predicts disease groups or autoantibodies in patients with idiopathic inflammatory myopathies (IIMs) using muscle MRI... (Observational Study)
Observational Study
To develop a machine learning (ML) model that predicts disease groups or autoantibodies in patients with idiopathic inflammatory myopathies (IIMs) using muscle MRI radiomics features. Twenty-two patients with dermatomyositis (DM), 14 with amyopathic dermatomyositis (ADM), 19 with polymyositis (PM) and 19 with non-IIM were enrolled. Using 2D manual segmentation, 93 original features as well as 93 local binary pattern (LBP) features were extracted from MRI (short-tau inversion recovery [STIR] imaging) of proximal limb muscles. To construct and compare ML models that predict disease groups using each set of features, dimensional reductions were performed using a reproducibility analysis by inter-reader and intra-reader correlation coefficients, collinearity analysis, and the sequential feature selection (SFS) algorithm. Models were created using the linear discriminant analysis (LDA), quadratic discriminant analysis (QDA), support vector machine (SVM), k-nearest neighbors (k-NN), random forest (RF) and multi-layer perceptron (MLP) classifiers, and validated using tenfold cross-validation repeated 100 times. We also investigated whether it was possible to construct models predicting autoantibody status. Our ML-based MRI radiomics models showed the potential to distinguish between PM, DM, and ADM. Models using LBP features provided better results, with macro-average AUC values of 0.767 and 0.714, accuracy of 61.2 and 61.4%, and macro-average recall of 61.9 and 59.8%, in the LDA and k-NN classifiers, respectively. In contrast, the accuracies of radiomics models distinguishing between non-IIM and IIM disease groups were low. A subgroup analysis showed that classification models for anti-Jo-1 and anti-ARS antibodies provided AUC values of 0.646-0.853 and 0.692-0.792, with accuracy of 71.5-81.0 and 65.8-78.3%, respectively. ML-based TA of muscle MRI may be used to predict disease groups or the autoantibody status in patients with IIM and is useful in non-invasive assessments of disease mechanisms.
Topics: Adult; Aged; Antibodies, Antinuclear; Antigens, Ly; Biopsy; Dermatomyositis; Diagnosis, Differential; Female; Humans; Image Interpretation, Computer-Assisted; Machine Learning; Magnetic Resonance Imaging; Male; Middle Aged; Muscles; Polymyositis; ROC Curve; Reproducibility of Results; Retrospective Studies; Urokinase-Type Plasminogen Activator
PubMed: 33972636
DOI: 10.1038/s41598-021-89311-3 -
Rheumatology International Jun 2021Anti-Melanoma Differentiation-Associated gene 5 (MDA-5) Dermatomyositis (MDA5, DM) is a recently identified subtype of myositis characteristically associated with... (Review)
Review
Anti-Melanoma Differentiation-Associated gene 5 (MDA-5) Dermatomyositis (MDA5, DM) is a recently identified subtype of myositis characteristically associated with Rapidly Progressive Interstitial Lung Disease (RP-ILD) and unique cutaneous features. We reviewed PubMed, SCOPUS and Web of Science databases and selected 87 relevant articles after screening 1485 search results, aiming to gain a better understanding of the pathophysiology, clinical features, diagnosis, and treatment approaches of anti-MDA-5 DM described in the literature. The etiopathogenesis is speculatively linked to an unidentified viral trigger on the background of genetic predisposition culminating in an acquired type I interferonopathy. The clinical phenotype is highly varied in different ethnicities, with new clinical features having been recently described, expanding the spectrum of cases that should raise the suspicion of anti-MDA-5 DM. Unfortunately, the diagnosis is frequently missed despite excessive mortality, calling for wider awareness of suspect symptoms. RP ILD is the major determinant of survival, treatment being largely based on observational studies with recent insights into aggressive combined immunosuppression at the outset.
Topics: COVID-19; Dermatomyositis; Disease Progression; Exanthema; Female; Humans; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Male; Prevalence; SARS-CoV-2
PubMed: 33774723
DOI: 10.1007/s00296-021-04819-1 -
Journal of Medical Economics Jul 2016Background Dermatomyositis and polymyositis (DM/PM) are inflammatory myopathies characterized by muscle inflammation/weakness. Patients with DM/PM have a reduced...
Background Dermatomyositis and polymyositis (DM/PM) are inflammatory myopathies characterized by muscle inflammation/weakness. Patients with DM/PM have a reduced quality-of-life and are at an increased risk for several comorbidities. Studies have assessed the incidence and prevalence of DM/PM; however, no study has estimated the burden of the diseases in terms of both healthcare resource utilization (HCRU) and work loss incurred by patients. Objective To provide a comprehensive, current estimate of the annual HCRU and work loss in DM/PM patients in the US. Methods All patients (aged 18-64 years) with a first diagnosis of DM/PM between January 1, 1998 and March 31, 2014 ('index date') were selected from a de-identified privately-insured administrative claims database. DM/PM patients were required to have continuous health-plan enrollment 12 months prior to and following their index date. Propensity-score (1:1) matching of DM/PM patients with non-DM/PM controls was carried out based on a logistic regression of demographic characteristics, comorbidities, costs, and HCRU to control for these confounding factors. Burden of HCRU and work loss (disability days and medically-related absenteeism) were compared between the matched DM/PM and the non-DM/PM cohorts over the 12-month period after the index date ('outcome period'). Results Of the 2617 DM/PM patients that met sample selection criteria, 2587 (98.9%) were matched with a non-DM/PM control. During the outcome period, DM/PM patients had significantly increased HCRU across places of service, including 44% more inpatient admissions (3.6 vs 2.5, p < 0.001), increased visits with specialists such as rheumatologists, neurologists and physical therapists, and filled 4.7 more prescriptions (32.2 vs 27.5, p < 0.001) than matched control patients. The increased HCRU led to significantly more medically-related work loss among DM/PM patients than matched controls (p < 0.001). Conclusions DM/PM imposes a substantial increase in healthcare resource use and is associated with statistically significantly greater work loss in the first year following diagnosis.
Topics: Absenteeism; Adolescent; Adult; Comorbidity; Cost of Illness; Dermatomyositis; Female; Health Services; Humans; Insurance Claim Review; Insurance, Health; Logistic Models; Male; Middle Aged; Polymyositis; Private Sector; Propensity Score; Socioeconomic Factors; Young Adult
PubMed: 26850074
DOI: 10.3111/13696998.2016.1151433 -
Scientific Reports Feb 2021Our study aimed to investigate the incidence, risk factors and time to occurrence of malignancy in patients with dermatomyositis (DM) and polymyositis (PM). The...
Our study aimed to investigate the incidence, risk factors and time to occurrence of malignancy in patients with dermatomyositis (DM) and polymyositis (PM). The electronic medical records of 1100 patients with DM and 1164 patients with PM were studied between January 2001 and May 2019. Malignancies after myositis were diagnosed in 61 (5.55%) patients with DM and 38 (3.26%) patients with PM. The cumulative incidence of malignancies in patients with DM were significantly higher than patients with PM (hazard ratio = 1.78, log-rank p = 0.004). Patients with DM had a greater risk of developing malignancy than those with PM at 40-59 years old (p = 0.01). Most malignancies occurred within 1 year after the initial diagnosis of DM (n = 35; 57.38%). Nasopharyngeal cancer (NPC) was the most common type of malignancy in patients with DM (22.95%), followed by lung, and breast cancers. In patients with PM, colorectal, lung and hepatic malignancies were the top three types of malignancy. The risk factors for malignancy included old age (≥ 45 years old) and low serum levels of creatine phosphokinase (CPK) for patients with DM and male sex and low serum levels of CPK for patients with PM. Low serum levels of CPK in patients with myositis with malignancy represented a low degree of muscle destruction/inflammation, which might be attributed to activation of the PD-L1 pathway by tumor cells, thus inducing T-cell dysfunction mediating immune responses in myofibers. A treatment and follow-up algorithm should explore the occurrence of malignancy in different tissues and organs and suggested annual follow-ups for at least 5.5 years to cover the 80% cumulative incidence of malignancy in patients with DM and PM.
Topics: Adult; Aged; Aged, 80 and over; Comorbidity; Dermatomyositis; Diagnosis, Differential; Female; Humans; Incidence; Male; Middle Aged; Polymyositis; Public Health Surveillance; Registries; Risk Assessment; Risk Factors; Taiwan; Young Adult
PubMed: 33633147
DOI: 10.1038/s41598-021-83729-5 -
Tomography (Ann Arbor, Mich.) Mar 2024(1) Background: The intravoxel incoherent motion (IVIM) model can provide information about both molecular diffusion and blood flow for the evaluation of skeletal muscle...
(1) Background: The intravoxel incoherent motion (IVIM) model can provide information about both molecular diffusion and blood flow for the evaluation of skeletal muscle inflammation. MRI-based fat quantification is advantageous for assessing fat infiltration in skeletal muscle. (2) Purpose: We aimed to quantitatively measure various parameters associated with IVIM diffusion-weighted imaging (DWI) and fat quantification in the muscles of patients with polymyositis and dermatomyositis using magnetic resonance imaging and to investigate the relationship between these parameters and electromyography (EMG) findings. (3) Material and methods: Data were retrospectively evaluated for 12 patients with polymyositis and dermatomyositis who underwent thigh MRI, including IVIM-DWI and fat quantification. The IVIM-derived parameters included the pure diffusion coefficient (D), pseudodiffusion coefficient (D*), and perfusion fraction (). Fat fraction values were assessed using the six-point Dixon technique. Needle EMG was performed within 9 days of the MRI. (4) Results: The values (19.02 ± 4.87%) in muscles with pathological spontaneous activity on EMG were significantly higher than those (14.60 ± 5.31) in muscles without pathological spontaneous activity ( < 0.027). There were no significant differences in D, D*, ADC, or fat fraction between muscles with and without pathologic spontaneous activity. Significant negative correlations were observed between fat fraction and amplitude ( = -0.402, < 0.015) and between fat fraction and duration ( = -0.360, < 0.031). (5) Conclusion: The current study demonstrates that IVIM-DWI and fat quantification using 3.0 T MRI may aid in predicting EMG findings in patients with polymyositis and dermatomyositis and promote the pathophysiological study of idiopathic inflammatory myopathies.
Topics: Humans; Electromyography; Dermatomyositis; Retrospective Studies; Diffusion Magnetic Resonance Imaging; Polymyositis; Myositis
PubMed: 38535771
DOI: 10.3390/tomography10030029 -
Saudi Medical Journal May 2021We report a case involving a 73-year-old Saudi man diagnosed with dermatomyositis who subsequently developed Kaposi's sarcoma one month later. He had difficulty in...
We report a case involving a 73-year-old Saudi man diagnosed with dermatomyositis who subsequently developed Kaposi's sarcoma one month later. He had difficulty in rising from a chair and increased leg weakness while climbing stairs or walking. He was unable to comb his hair and had greater dysphagia with liquids than with solid foods. Laboratory tests showed elevated liver enzyme and creatine kinase levels. Right quadriceps muscle biopsy indicated inflammatory myopathy, which was consistent with adult dermatomyositis. We administered 1-g/day methylprednisolone intravenously for 3 days, followed by 60-mg oral prednisolone daily, which led to significant improvements in his muscle strength and dysphagia symptoms. The creatine phosphokinase level normalized. Computed tomography and endoscopic examinations did not reveal any evidence of an underlying malignancy. The patient was discharged and underwent close monitoring at a rheumatology clinic. We subsequently added 50-mg oral azathioprine daily to the treatment regimen. At one month after emergency room presentation, multiple new dusky violaceous macules and plaques appeared on his chest, face, upper back, neck, and upper limbs. A biopsy of one of these lesions showed findings consistent withKaposi's sarcoma. He was referred to an oncology center for chemotherapy, following which his skin lesions significantly regressed. Dermatomyositis may be considered a paraneoplastic syndrome of Kaposi's sarcoma.
Topics: Aged; Biopsy; Dermatomyositis; Humans; Male; Prednisolone; Sarcoma, Kaposi
PubMed: 33896788
DOI: 10.15537/smj.2021.42.5.20200583 -
PloS One 2016Interstitial lung disease (ILD) is an extramuscular manifestation that results in increased morbidity and mortality from polymyositis (PM) and dermatomyositis (DM). The... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVES
Interstitial lung disease (ILD) is an extramuscular manifestation that results in increased morbidity and mortality from polymyositis (PM) and dermatomyositis (DM). The aim of this study was to systematically evaluate risk factors associated with the development of ILD in PM/DM.
METHODS
Observational studies were identified from searching PubMed, Medline, Embase, and the Cochrane Library. Pooled odds ratios (ORs) or standardized mean differences (SMDs) and corresponding 95% confidence intervals (CIs) were obtained for the relationships between risk factors and ILD in PM/DM using either fixed- or random-effects models, whichever were appropriate. Heterogeneity tests, sensitivity analyses, and publication bias assessments were also performed.
RESULTS
Twenty-three studies were selected for a meta-analysis that included 834 patients and 1245 control subjects. Risk factors that may have increased the risk of developing ILD in PM/DM patients included older age at diagnosis (SMD, 0.35; 95% CI, 0.18-0.52; P < 0.0001), arthritis/arthralgia (OR, 3.17; 95% CI, 1.99-5.04; P < 0.00001), fever (OR, 2.31; 95% CI, 1.42-3.76; P = 0.0007), presence of anti-Jo-1 antibodies (OR, 3.34; 95% CI, 2.16-5.16; P < 0.00001), elevated erythrocyte sedimentation rate (ESR; SMD, 0.48; 95% CI, 0.32-0.64; P < 0.00001), presence of anti-MDA5 antibodies (OR, 18.26; 95% CI, 9.66-34.51; P < 0.00001), and elevated C-reactive protein level (CRP; OR, 3.50; 95% CI, 1.48-8.28; P = 0.004). Meanwhile, malignancy (OR, 0.36; 95% CI, 0.18-0.72; P = 0.004) reduced the risk of developing ILD in PM/DM patients.
CONCLUSION
Our meta-analysis results suggest that the association between PM/DM and ILD may be due to such risk factors as older age at diagnosis, arthritis/arthralgia, fever, presence of anti-Jo-1 antibodies, elevated ESR, presence of anti-MDA5 antibodies, and elevated CRP level, while malignancy was associated with a reduced risk of developing ILD. Thus, these variables may be used to guide screening processes for ILD in patients with PM/DM.
Topics: Demography; Dermatomyositis; Female; Humans; Lung Diseases, Interstitial; Male; Polymyositis; Publication Bias; Risk Factors
PubMed: 27171228
DOI: 10.1371/journal.pone.0155381 -
The British Journal of Dermatology May 2019Diagnostic criteria are used to identify a patient having a disease in a clinical setting, whereas classification criteria create a well-defined population for research... (Review)
Review
BACKGROUND
Diagnostic criteria are used to identify a patient having a disease in a clinical setting, whereas classification criteria create a well-defined population for research purposes. The diagnosis and classification of amyopathic dermatomyositis (ADM) have not been recognized by most existing criteria for idiopathic inflammatory myopathies (IIMs). To address this, several criteria were proposed to define ADM either as a distinct disease entity or as a subset of the spectrum of IIMs.
OBJECTIVES
To discuss the diagnosis and classification of ADM and to assesses the available criteria in identifying cases of ADM and/or distinguishing it from dermatological mimickers such as lupus erythematosus.
METHODS
We conducted an extensive literature search using the PubMed database from June 2016 to August 2018, using the search terms 'amyopathic dermatomyositis', 'diagnosis' and 'classification'.
RESULTS
The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) criteria, which are the only validated classification criteria for adult and juvenile IIM and their major subgroups, include three cutaneous items (Göttron sign, Göttron papules, heliotrope rash) to be able to classify ADM. This international and multispecialty effort is a huge step forward in the classification of skin-predominant disease in dermatomyositis. However, about 25% of the population with ADM do not meet two out of the three skin features and are misdiagnosed or classified as having a different disease entity, most commonly lupus erythematosus.
CONCLUSIONS
These gaps rationalize the continuous assessment and improvement of existing criteria and/or the development of validated, separate and skin-focused criteria for DM.
Topics: Dermatology; Dermatomyositis; Diagnosis, Differential; History, 20th Century; History, 21st Century; Humans; Lupus Erythematosus, Cutaneous; Rheumatology; Skin
PubMed: 30561064
DOI: 10.1111/bjd.17536 -
Journal of Investigative Medicine High... 2021Psoriatic arthritis is an inflammatory arthritis, most commonly occurring several years after the onset of psoriasis. Psoriatic arthritis is associated with many...
Psoriatic arthritis is an inflammatory arthritis, most commonly occurring several years after the onset of psoriasis. Psoriatic arthritis is associated with many comorbidities, including diabetes mellitus, nonalcoholic fatty liver disease, fibromyalgia, and cardiovascular disease. Dermatomyositis is an inflammatory myopathy primarily affecting the skin and muscles. As per literature review, cases of psoriasis and dermatomyositis have been reported. In most published cases, the courses of these diseases develop independently. This is the case of a 45-year-old woman initially diagnosed with psoriatic arthritis who developed concurrent dermatomyositis. The methods used were PubMed search and UpToDate search.
Topics: Arthritis, Psoriatic; Dermatomyositis; Humans; Middle Aged
PubMed: 34911389
DOI: 10.1177/23247096211057702 -
Reumatismo Dec 2023Idiopathic immune myopathies (IIMs) are autoimmune diseases caused by immune-mediated muscle damage. The etiology remains unclear. Epidemiological and experimental...
Idiopathic immune myopathies (IIMs) are autoimmune diseases caused by immune-mediated muscle damage. The etiology remains unclear. Epidemiological and experimental studies, both in animals and humans, hint at viruses as major environmental factors able to trigger aberrant immune responses through many different mechanisms. However, only a few cases of either dermatomyositis or polymyositis following a specific viral infection have been reported in the literature. The objective of this study is to describe the clinical features and the treatment strategy of 2 cases of polymyositis developing shortly after chickenpox and mumps, respectively, and to review the existing literature on the topic. The clinical records of the 2 patients suspected to have developed inflammatory myositis following a viral infection were reviewed. Their clinical history, main laboratory findings, and treatment outcome are presented here. Moreover, a literature search was performed in the PubMed and MEDLINE databases to identify reports describing the association between viral infections and IIMs in patients aged ≥18. The 2 patients reported here developed polymyositis shortly after chickenpox and mumps, respectively, suggesting a causal role for viruses in triggering autoimmunity. Only a few reports published between 1990 and 2020 were found in the literature, possibly linking infections to myositis development. Intravenous immunoglobulin and rituximab were effective for the treatment of viral-triggered polymyositis.
Topics: Adult; Humans; Autoimmune Diseases; Chickenpox; Dermatomyositis; Mumps; Myositis; Polymyositis
PubMed: 38115780
DOI: 10.4081/reumatismo.2023.1562