[Magnetic resonance imaging T2 mapping could reflect disease status in patients with dermatomyositis or polymyositis].

This study aimed to explore the value of magnetic resonance imaging (MRI) T2 mapping in the assessment of dermatomyositis (DM) and polymyositis (PM). Thirty-three confirmed cases (myosin group) and eight healthy volunteers (healthy control group) at the Department of Rheumatology and Immunology, the First Affiliated Hospital of Kunming Medical University, from October 2016 to December 2017, were collected and analyzed. Multiple parameters of the myosin group were quantified, including creatine kinase (CK), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement C3, and complement C4. Disease status was evaluated using a panel of tools: myositis disease activity assessment tool-muscle (MDAAT-muscle), myositis disease activity assessment tool-whole (MDAAT-all), health assessment questionnaire (HAQ), medical outcomes study health survey short form-36 item (SF-36), hand muscle strength test (MMT-8) score, and MRI T2 mapping of muscle (22 muscles in the pelvis and thighs) T2 values. The results showed that in the myositis group, the measurements for CK, ESR, CRP, complement C3, and complement C4 were 457.2 (165.6, 1 229.2) IU/L, 20 (10, 42) mm/1h, 3.25 (2.38, 10.07) mg/L, 0.90 (0.83, 1.06) g/L, and 0.18 (0.14, 0.23) g/L, respectively. The scores for MMT-8, MDAAT-muscle, MDAAT-all, HAQ, and SF-36 were 57.12±16.23, 5.34 (4.00, 6.00), 34.63±12.62, 1.55 (0.66, 2.59), and 44.66±7.98, respectively. T2 values were significantly higher in all 22 muscles of the pelvis and thighs of patients with DM or PM compared with the healthy controls [(54.99±11.60)ms vs. (36.62±1.66)ms, P<0.001], with the most severe lesions in the satrorius, iliopsoas, piriformis, gluteus minimus, and gluteus medius muscles. The total muscle T2 value in the myositis group was positively correlated with CK, MDAAT-muscle, MDAAT-all, and HAQ (r=0.461, 0.506, 0.347, and 0.510, respectively, all P<0.05). There was a negative correlation between complement C4, SF-36, and MMT-8 scores (r=-0.424, -0.549, and -0.686, respectively, all P<0.05). Collectively, the findings from this study suggest that MRI T2 mapping can objectively reflect the disease status of DM and PM.

Value of radiomics-based automatic grading of muscle edema in polymyositis/dermatomyositis based on MRI fat-suppressed T2-weighted images.

BACKGROUND: The precise and objective assessment of thigh muscle edema is pivotal in diagnosing and monitoring the treatment of dermatomyositis (DM) and polymyositis (PM). PURPOSE: Radiomic features are extracted from fat-suppressed (FS) T2-weighted (T2W) magnetic resonance imaging (MRI) of thigh muscles to enable automatic grading of muscle edema in cases of polymyositis and dermatomyositis. MATERIAL AND METHODS: A total of 241 MR images were analyzed and classified into five levels using the Stramare criteria. The correlation between muscle edema grading and T2-mapping values was assessed using Spearman's correlation. The dataset was divided into a 7:3 ratio of training (168 samples) and testing (73 samples). Thigh muscle boundaries in FS T2W images were manually delineated with 3D-Slicer. Radiomics features were extracted using Python 3.7, applying Z-score normalization, Pearson correlation analysis, and recursive feature elimination for reduction. A Naive Bayes classifier was trained, and diagnostic performance was evaluated using receiver operating characteristic (ROC) curves and comparing sensitivity and specificity with senior doctors. RESULTS: A total of 1198 radiomics parameters were extracted and reduced to 18 features for Naive Bayes modeling. In the testing set, the model achieved an area under the ROC curve of 0.97, sensitivity of 0.85, specificity of 0.98, and accuracy of 0.91. The Naive Bayes classifier demonstrated grading performance comparable to senior doctors. A significant correlation (r = 0.82, P <0.05) was observed between Stramare edema grading and T2-mapping values. CONCLUSION: The Naive Bayes model, utilizing radiomics features extracted from thigh FS T2W images, accurately assesses the severity of muscle edema in cases of PM/DM.

Quantitative whole-body muscle MRI in idiopathic inflammatory myopathies including polymyositis with mitochondrial pathology: indications for a disease spectrum.

OBJECTIVE: Inflammatory myopathies (IIM) include dermatomyositis (DM), sporadic inclusion body myositis (sIBM), immune-mediated necrotizing myopathy (IMNM), and overlap myositis (OLM)/antisynthetase syndrome (ASyS). There is also a rare variant termed polymyositis with mitochondrial pathology (PM-Mito), which is considered a sIBM precursor. There is no information regarding muscle MRI for this rare entity. The aim of this study was to compare MRI findings in IIM, including PM-Mito. METHODS: This retrospective analysis included 41 patients (7 PM-Mito, 11 sIBM, 11 PM/ASyS/OLM, 12 IMNM) and 20 healthy controls. Pattern of muscle involvement was assessed by semiquantitative evaluation, while Dixon method was used to quantify muscular fat fraction. RESULTS: The sIBM typical pattern affecting the lower extremities was not found in the majority of PM-Mito-patients. Intramuscular edema in sIBM and PM-Mito was limited to the lower extremities, whereas IMNM and PM/ASyS/OLM showed additional edema in the trunk. Quantitative assessment showed increased fat content in sIBM, with an intramuscular proximo-distal gradient. Similar changes were also found in a few PM-Mito- and PM/ASyS/OLM patients. In sIBM and PM-Mito, mean fat fraction of several muscles correlated with clinical involvement. INTERPRETATION: As MRI findings in patients with PM-Mito relevantly differed from sIBM, the attribution of PM-Mito as sIBM precursor should be critically discussed. Some patients in PM/ASyS/OLM and PM-Mito group showed MR-morphologic features predominantly observed in sIBM, indicative of a spectrum from PM/ASyS/OLM toward sIBM. In some IIM subtypes, MRI may serve as a biomarker of disease severity.

Thymus variants on imaging of patients with primary Sjögren's syndrome and polymyositis/dermatomyositis: clinical and immunological significance.

We investigated the presence of radiographic thymus variants using a scoring system and examined their association with clinical and immunological features in primary Sjögren's syndrome (pSS) and polymyositis/dermatomyositis (PM/DM) patients. Cases of 72 patients with pSS and 47 with PM/DM were randomly selected from all visitors to our department who received chest CT scanning, excluding those with thymoma or thymic cyst, or age <30 years. We quantitatively interpreted and assessed thymus size and attenuation score in axial CT images. Thymic enlargement was identified in 16 (22.2%) pSS and 14 (29.8%) PM/DM patients. A thymus attenuation score ≥ 2 was seen in 11 (15.3%) pSS and 9 (19.1%) PM/DM patients. Thymic enlargement showed a significant association with the titre of rheumatoid factor in PM/DM patients. Thymic enlargement and score showed a significant association with body weight in pSS patients. Radiographic thymus variants are often observed in pSS and PM/DM patients, particularly in cases of PM/DM, and may suggest the role of an abnormal immune response in their pathogenesis.

Clinical and Radiological Features of Interstitial Lung Diseases Associated with Polymyositis and Dermatomyositis.

Polymyositis and dermatomyositis are autoimmune idiopathic systemic inflammatory diseases, characterized by various degrees of muscle inflammation and typical cutaneous lesions-the latter found in dermatomyositis. The underlying pathogenesis is characterized by a high level of uncertainty, and recent studies suggest diseases may have different immunopathological mechanisms. In polymyositis, components of the cellular immune system are involved, whereas in dermatomyositis, the pathogenesis is mainly mediated by the humoral immune response. The interstitial lung disease occurs in one-third of polymyositis and dermatomyositis patients associated with worse outcomes, showing an estimated excess mortality rate of around 40%. Lung involvement may also appear, such as a complication of muscle weakness, mainly represented by aspiration pneumonia or respiratory insufficiency. The clinical picture is characterized, in most cases, by progressive dyspnea and non-productive cough. In some cases, hemoptysis and chest pain are found. Onset can be acute, sub-acute, or chronic. Pulmonary involvement could be assessed by High Resolution Computed Tomography (HRCT), which may identify early manifestations of diseases. Moreover, Computed Tomography (CT) appearances can be highly variable depending on the positivity of myositis-specific autoantibodies. The most common pathological patterns include fibrotic and cellular nonspecific interstitial pneumonia or organizing pneumonia; major findings observed on HRCT images are represented by consolidations, ground-glass opacities, and reticulations. Other findings include honeycombing, subpleural bands, and traction bronchiectasis. In patients having Anti-ARS Abs, HRCT features may develop with consolidations, ground glass opacities (GGOs), and reticular opacities in the peripheral portions; nonspecific interstitial pneumonia or nonspecific interstitial pneumonia mixed with organizing pneumonia have been reported as the most frequently encountered patterns. In patients with anti-MDA5 Abs, mixed or unclassifiable patterns are frequently observed at imaging. HRCT is a sensitive method that allows one not only to identify disease, but also to monitor the effectiveness of treatment and detect disease progression and/or complications; however, radiological findings are not specific. Therefore, aim of this pictorial essay is to describe clinical and radiological features of interstitial lung diseases associated with polymyositis and dermatomyositis, emphasizing the concept that gold standard for diagnosis and classification-should be based on a multidisciplinary approach.

Myocardial involvement characteristics by cardiac MR imaging in patients with polymyositis and dermatomyositis.

OBJECTIVE: Myocardial involvement is frequently observed in PM and DM but typically remains subclinical. This study aimed to investigate characteristics of myocardial involvement and compare differences between patients with PM and DM by cardiac MR (CMR) imaging. METHODS: From March 2017 to December 2019, a total of 17 PM and 27 DM patients were enrolled in this retrospective study. In all patients, clinical assessment and CMR examination were performed. CMR parameters, including left ventricular (LV) morphologic and functional parameters, and CMR tissue characterization imaging parameters, such as native T1, T2, extracellular volume (ECV) and late gadolinium enhancement, were analysed. RESULTS: In patients in both PM and DM groups, elevated global native T1 and ECV values were observed. Global ECV values were higher in the PM group when compared with the DM group (33.24 ± 2.97% vs 30.36 ± 4.20%; P = 0.039). Furthermore, patients in the PM and DM groups showed a different positive segment distribution of late gadolinium enhancement, native T1 and ECV, whereas the number of positive segments in PM patients was greater compared with that in DM patients. No significant differences in LV morphological and functional parameters were observed between patients in PM and DM groups, and most were in normal range. CONCLUSION: CMR tissue characterization imaging could detect early myocardial involvement in PM and DM patients without overt LV dysfunction. Furthermore, characteristics of myocardial involvement were different between PM and DM patients with more serious myocardial involvement seen in PM patients.

Role of MRI in idiopathic inflammatory myopathies: a review article.

Idiopathic inflammatory myopathies are a rare heterogeneous group of chronic, autoimmune conditions characterized by the slow, progressive weakness of the skeletal muscles and inflammatory infiltrates in the muscle tissue. The predominant role of magnetic resonance imaging (MRI) in myositis imaging is to assess disease activity and to identify the target site for biopsy. Its role in phenotyping the disease is less explored. The aim of the present review was to examine the role of MRI in differentiating between the common inflammatory myopathies, i.e. dermatomyositis, polymyositis, and sporadic inclusion body myositis, and to describe the specific spectrum of MRI findings in various inflammatory myopathies.

Conventional Radiography and Ultrasound Imaging of Rheumatic Diseases Affecting the Pediatric Population.

Juvenile idiopathic arthritis is the most frequent rheumatic disease in the pediatric population, followed by systemic lupus erythematosus, juvenile scleroderma syndromes, juvenile dermatomyositis, chronic recurrent multifocal osteomyelitis, and juvenile vasculopathies. The imaging approach to inflammatory connective tissue diseases in childhood has not changed dramatically over the last decade, with radiographs still the leading method for bony pathology assessment, disease monitoring, and evaluation of growth disturbances. Ultrasonography is commonly used for early detection of alterations within the intra- and periarticular soft tissues, assessing their advancement and also disease monitoring. It offers several advantages in young patients including nonionizing radiation exposure, short examination time, and high resolution, allowing a detailed evaluation of the musculoskeletal system for the features of arthritis, tenosynovitis, enthesitis, bursitis, myositis, as well as pathologies of the skin, subdermis, vessels, and fasciae. In this pictorial essay we discuss radiographic and ultrasound inflammatory features of autoimmune pediatric inflammatory arthropathies: juvenile idiopathic arthritis, lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis and polymyositis.

Semi-automated volumetry of MRI serves as a biomarker in neuromuscular patients.

BACKGROUND: Muscle MRI is of increasing importance for neuromuscular patients to detect changes in muscle volume, fat-infiltration, and edema. We developed a method for semi-automated segmentation of muscle MRI datasets. METHODS: An active contour-evolution algorithm implemented within the ITK-SNAP software was used to segment T1-weighted MRI, and to quantify muscle volumes of neuromuscular patients (n = 65). RESULTS: Semi-automated compared with manual segmentation was shown to be accurate and time-efficient. Muscle volumes and ratios of thigh/lower leg volume were lower in myopathy patients than in controls (P < .0001; P < .05). We found a decrease of lower leg muscle volume in neuropathy patients compared with controls (P < .01), which correlated with clinical parameters. In myopathy patients, muscle volume showed a positive correlation with muscle strength (rleft = 0.79, pleft < .0001). Muscle volumes were independent of body mass index and age. CONCLUSIONS: Our method allows for exact and time-efficient quantification of muscle volumes with possible use as a biomarker in neuromuscular patients.

Differential and quantitative neuroimaging characteristics of inclusion body myositis.

In clinical settings, it is often difficult to distinguish inclusion body myositis (IBM) from other neuromuscular diseases. In order to clarify clinically useful characteristics for making the differential diagnosis of IBM, we performed clinical, epidemiological, and neuroimaging analyses in patients with various types of neuromuscular disorders. We enrolled 333 patients with myopathy and 12 patients with amyotrophic lateral sclerosis (ALS) who had been hospitalized in our department from January 1, 1979, to December 31, 2018. Among them, 18 patients with IBM, 16 patients with polymyositis (PM), and 12 patients with ALS who showed equivalent severity of muscle weakness in their lower limbs underwent the quantitative neuroimaging analysis using lower limb CT and clinical assessment. Patients with IBM exhibited significantly greater muscular degeneration in the rectus femoris, vastus, sartorius, adductor, anterior calf, and medial gastrocnemius muscles than those with PM or ALS. The ratio of the remaining muscle area of the quadriceps relative to that of the hamstrings and the duration from onset to CT imaging were negatively correlated in patients with IBM, indicating that the anterior thigh muscles were preferentially affected over the posterior muscles. Characteristic muscular degeneration in the lower limbs on CT imaging may aid for making the diagnosis of IBM.

18F-FDG PET/CT in a Patient With Thymoma-Associated Paraneoplastic Polymyositis.

A 43-year-old woman presented with progressive muscle weakness and myalgia for 1 month. Laboratory tests revealed the elevation of myogenic enzymes. MRI of the thighs demonstrated a pattern of edema for inflammatory myopathy. Electromyography showed diffuse myopathic disorder, and muscle biopsy indicated polymyositis. F-FDG PET/CT was performed to investigate the potential paraneoplastic syndrome and revealed a soft tissue lesion with F-FDG avidity in the right anterior mediastinum, as well as diffusely increased activity throughout the body muscles. The patient's symptoms and biochemical abnormalities were gradually relieved after the tumor resection, and the histopathology indicated a type AB thymoma.

18F-FDG PET/CT in Graft Versus Host Disease-Associated Polymyositis.

Graft versus host disease (GVHD) is the most frequent and serious complication of allogenic hematopoietic stem cell transplant. Polymyositis is a rare neuromuscular manifestation of GVHD, which often responds well to corticosteroid and immunosuppression therapy. We present a case of a 38-year-old man with a known case of mixed-phenotype acute leukemia after hematopoietic stem cell transplant presenting with GVHD-associated polymyositis. F-FDG PET/CT done in this case not only helped in the detection of the muscle involvement but also helped in obtaining precise muscle sample for histopathological diagnosis using PET-guided biopsy.

PET Scan: Nuclear Medicine Imaging in Myositis.

PURPOSE OF REVIEW: Positron emission tomography (PET) combined with computed tomography (CT) has proven useful as a cancer screening technique in patients with inflammatory myopathy, mainly dermatomyositis. In this review, we focus on advances in this direction and other potential applications of PET/CT in patients with inflammatory myopathy. RECENT FINDINGS: Cancer screening by PET/CT seems suitable and cost-effective in patients with myositis. It has also shown value as a hybrid technique for diagnosing myositis versus controls and could be of interest for differentiating between polymyositis and sporadic inclusion body myositis. Quantification of muscle activity by PET/CT seems reliable. Preliminary data suggest that it could also be used to diagnose and measure the activity of the disease in the lung. PET/CT should be in the toolbox of physicians managing patients with myositis. The multiple applications of PET/CT include its value for cancer screening, measuring the activity of the disease in muscle, and helping to differentiate between myositis phenotypes. The possibility to diagnose and monitor inflammatory lung activity remains to be demonstrated in well-designed studies.

Radiographic patterns of muscle involvement in the idiopathic inflammatory myopathies.

INTRODUCTION: This study assesses the burden, distribution, and evolution of muscle inflammation and damage on MRI among subtypes of idiopathic inflammatory myopathy (IIM). METHODS: Musculoskeletal MRIs performed in 66 patients with IIM and 10 patients with non-IIM between 2009 and 2016 were retrospectively graded for muscle edema, fatty replacement (FR), and atrophy. RESULTS: Immune-mediated necrotizing myopathy (IMNM) patients had severe and extensive lower limb muscle edema, FR, and atrophy. The pelvic muscles and adductors were significantly more affected than in patients with dermatomyositis and polymyositis. Inclusion body myositis (IBM) was characterized by marked anterior thigh involvement, which stabilized or progressed at follow-up imaging. Atrophy and FR grades improved over time in some non-IBM IIM patients. DISCUSSION: Patients with IMNM and IBM have characteristic patterns of muscle MRI abnormalities that may allow them to be differentiated radiologically from other IIM subtypes. Muscle damage in non-IBM IIM may be reversible.

Quantitative T2 mapping accelerated by GRAPPATINI for evaluation of muscles in patients with myositis.

OBJECTIVE: Dermatomyositis (DM) and polymyositis (PM) make up the largest group of potentially treatable myopathies and require early diagnosis. This study investigates whether the edema of thigh muscles in DM/PM can be quantitatively assessed by a novel accelerated T2 mapping technique-GRAPPATINI. METHODS: Three conventional MR sequences and GRAPPATINI accelerated T2 mapping of bilateral thighs from 20 patients (7 DM and 13 PM) and 10 healthy volunteers were prospectively carried out on a 3 T MR scanner. Afterwards, T2 values of 477 thigh muscles from the patients and the healthy controls were manually measured. In addition, the correlations between T2 values and serum muscle enzymes in patients were also analyzed. RESULTS: The new GRAPPATINI technique made quantitative T2 mapping of bilateral thighs feasible with a scanning time of only 2 min 18 s. Moreover, GRAPPATINI-generated T2 values of muscles from patients were markedly higher than those from healthy subjects (p < 0.001). GRAPPATINI accelerated T2 mapping appeared a more sensitive technique in that some DM/PM muscles appearing normal per conventional MRI had increased T2 relaxation time. Furthermore, GRAPPATINI-generated T2 values of DM/PM thigh muscles positively correlated with serum enzyme levels (p < 0.001), which reflected the severity of myopathy. CONCLUSION: GRAPPATINI can significantly shorten acquisition time of T2 mapping and may potentially be applied clinically in DM and PM. ADVANCES IN KNOWLEDGE: GRAPPATINI acceleration makes T2 mapping feasible in clinical practice in providing quantitative information regarding thigh muscle inflammation in DM and PM.

An Anti-PM/Scl-75 Antibody-positive Japanese Woman Who Developed Inflammatory Myopathy.

A 69-year-old Japanese woman presented with mild muscle weakness of the neck and symmetrical proximal parts of the upper and lower limbs. Laboratory tests, needle electromyography, and a muscle biopsy revealed inflammatory myopathy with an apparent clinical classification of polymyositis and positive findings for anti-PM/Scl-75 antibody. This antibody is rare among Japanese populations, and most Japanese patients with the antibody are not classified with the inflammatory myopathy seen in polymyositis. The muscle biopsy also showed marked necrotic and regenerative fibers. We need to collectively investigate patients with the potential to develop this disease, and to identify any unique characteristics for Asian populations, including Japanese.

Diagnostic Imaging of Inflammatory Myopathies: New Concepts and a Radiological Approach.

PURPOSE OF REVIEW: The purpose of this review article is to highlight the current role of diagnostic imaging in the assessment of inflammatory myopathies. RECENT FINDINGS: Recent research demonstrates that imaging plays an important role in evaluating patients with symptoms of an inflammatory myopathy. In general, MRI is the pivotal imaging modality for assessing inflammatory myopathies, revealing precise anatomic details because of changes in the signal intensity of the muscles. Whole-body MR imaging has become increasingly important over the last several years. US is also a valuable imaging modality for scanning muscles. Together with the clinical history, familiarity with the imaging features of inflammatory myopathies is essential for formulating an accurate diagnosis.