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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 -
Frontiers in Immunology 2024Clinical and prognostic features of Anti-MDA5-Positive Dermatomyositis (MDA5+ DM) are diverse. This study aimed to examine the peripheral immune cell profiles of...
OBJECTIVE
Clinical and prognostic features of Anti-MDA5-Positive Dermatomyositis (MDA5+ DM) are diverse. This study aimed to examine the peripheral immune cell profiles of patients with MDA5+ DM, identify disease endotypes related to the heterogeneous manifestations and prognosis, and guide individualized therapy regimen.
METHODS
This inpatient cohort included 123 patients with MDA5+ DM. Unsupervised hierarchical clustering analysis was used to derive disease endotypes from the circulating immune cell profiles on admission. Clinical symptoms, laboratory test results, inpatient treatments, and disease outcomes were then analyzed among the identified endotypes.
RESULTS
Three disease endotypes in MDA5+ DM were identified from peripheral immune cell profiles. Endotype1 had the highest percentages of CD4 T cells and monocytes, and the lowest percentage of neutrophils; Endotype2 had the highest percentage of B cells; Endotype3 had the highest percentage of CD8 T cells and NK cells. Clinical and prognostic heterogeneity of the endotypes were revealed. Endotype1 had the lowest 3-month mortality with the high incidence of periungual capillary changes. Endotype2 and Endotype3 had higher prevalence of rapidly progressive interstitial lung disease (RPILD) and mortality at 3 months than Endotype1. Meanwhile, Endotype3 had higher pneumocystis jiroveci and CMV viremia cases with significantly elevated of activated CD8 T cells and multiple cytokines than Endotype1.
CONCLUSION
Clustering analysis of peripheral immune cell profiles identified three different endotypes in MDA5+ dermatomyositis. Endotpye2 and 3 showed higher RPILD, 3-month mortality, pneumocystis jiroveci and CMV viremia.
Topics: Humans; Interferon-Induced Helicase, IFIH1; Dermatomyositis; CD8-Positive T-Lymphocytes; Viremia; Lung Diseases, Interstitial; Cytomegalovirus Infections
PubMed: 38533498
DOI: 10.3389/fimmu.2024.1349611 -
Anais Brasileiros de Dermatologia 2024Dermatomyositis (DM) is an infrequent disease subgroup of idiopathic inflammatory myopathies characterized by distinct skin lesions. However, high heterogeneity makes...
BACKGROUND
Dermatomyositis (DM) is an infrequent disease subgroup of idiopathic inflammatory myopathies characterized by distinct skin lesions. However, high heterogeneity makes clinical diagnosis and treatment of DM very challenging.
OBJECTIVES
Unsupervised classification in DM patients and analysis of key factors related to clinical outcomes.
METHODS
This retrospective study was conducted between 2017 and 2022 at the Department of Rheumatology, Xiangya Hospital, Central South University. 162 DM patients were enrolled for unsupervised hierarchical cluster analysis. In addition, we divided the clinical outcomes of DM patients into four subgroups: withdrawal, stabilization, aggravation, and death, and compared the clinical profiles amongst the subgroups.
RESULTS
Out of 162 DM patients, three clusters were defined. Cluster 1 (n = 40) was mainly grouped by patients with prominent muscular involvement and mild Interstitial Lung Disease (ILD). Cluster 2 (n = 72) grouped patients with skin rash, anti-Melanoma Differentiation Associated protein 5 positive (anti-MDA5), and Rapid Progressive Interstitial Lung Disease (RP-ILD). Cluster 3 (n = 50) grouped patients with the mildest symptoms. The proportion of death increased across the three clusters (cluster 3 < cluster 1 < cluster 2).
STUDY LIMITATIONS
The number of cases was limited for the subsequent construction and validation of predictive models. We did not review all skin symptoms or pathological changes in detail.
CONCLUSIONS
We reclassified DM into three clusters with different risks for poor outcome based on diverse clinical profiles. Clinical serological testing and cluster analysis are necessary to help clinicians evaluate patients during follow-up and conduct phenotype-based personalized care in DM.
Topics: Humans; Dermatomyositis; Female; Retrospective Studies; Male; Middle Aged; Phenotype; Adult; Cluster Analysis; Aged; Lung Diseases, Interstitial; Serologic Tests; Outcome Assessment, Health Care; Autoantibodies; Interferon-Induced Helicase, IFIH1; Severity of Illness Index
PubMed: 38522973
DOI: 10.1016/j.abd.2023.06.005 -
Cureus Feb 2024The sigmoid colon is an uncommon site for the origin of primary malignant lymphomas in the GI tract. Additionally, immunosuppressive agents, widely used in treating...
The sigmoid colon is an uncommon site for the origin of primary malignant lymphomas in the GI tract. Additionally, immunosuppressive agents, widely used in treating autoimmune diseases, have been associated with the induction of malignancies, including lymphoproliferative disorders. In this report, we present a rare case of GI perforation suggesting a link between immunosuppressive therapy, particularly tacrolimus treatment, and diffuse large B-cell lymphoma (DLBCL). A 75-year-old female patient presented with abdominal pain to our ER. She had a medical history of polymyositis and interstitial pneumonia, treated with the immunosuppressant tacrolimus. An abdominal CT scan revealed free gas in the abdominal cavity, leading to a diagnosis of GI perforation. The patient exhibited generalized peritonitis and underwent emergency surgery the same day. During surgery, a perforation in the sigmoid colon was identified, and a Hartmann procedure was performed. Postoperative pathology showed CD20+, CD30+, CD5-, CD10-, BCL6+, MUM1+, and MIB-1 LI of 50-60%. The diagnosis of DLBCL was confirmed, classified as EBV-positive diffuse large B-cell lymphoma, not otherwise specified (NOS), in the sigmoid colon, with positive EBER-ISH, LMP-1, and EBNA2 findings. Given her history of immunosuppressant use, she was categorized as having other iatrogenic immunodeficiency-associated lymphoproliferative disorders (OIIA-LPD), according to the WHO Classification of 2017. This case highlights the importance for clinicians to consider the risk of oncogenesis associated with the prolonged use of immunosuppressive agents.
PubMed: 38516454
DOI: 10.7759/cureus.54571 -
Anti-SAE dermatomyositis: clinical and histologic characteristics from a monocentric Italian cohort.Clinical and Experimental Rheumatology Feb 2024Multiple myositis-specific antibodies have been identified, each associated with different clinical subsets of dermatomyositis (DM). Anti-SAE associated DM is considered...
OBJECTIVES
Multiple myositis-specific antibodies have been identified, each associated with different clinical subsets of dermatomyositis (DM). Anti-SAE associated DM is considered the least studied subset. Our study aimed to evaluate the clinical and histological characteristics of DM patients with anti-SAE antibodies. As reference, patients with anti-Mi2 antibodies associated DM, representing a well-characterised subset, were analysed.
METHODS
We recorded data from our DM cohort in the INflammatory MYositis REgistry (INMYRE). Patients were divided into two groups: those positive for anti-SAE and those positive for anti-Mi2 antibodies. Clinical characteristics, including skin, muscle, and extra-muscular involvements, were recorded. Available muscle biopsies were compared between the two groups.
RESULTS
Of 92 DM patients, 10 (10.9%) were positive for anti-SAE and 17 (18.5%) for anti-Mi2. Anti-SAE positive DM patients showed classic DM findings but were characterised by a higher prevalence of skin itching (60% vs. 11.8%, p<0.01), shawl sign (40% vs. 5.9%, p<0.05) and lung involvement (30% vs. 0%, p<0.05) compared to anti-Mi2 positive patients. Furthermore, anti-SAE positive DM patients showed lower creatine kinase levels than those with anti-Mi2 (median [IQR]: 101 [58-647] vs. 1984 [974-3717], p<0.05) and a lower percentage of muscle fibre degeneration and necrosis (1.5%±1.7 vs. 5.9%±3.2, p<0.05) in muscle biopsies. No other differences were observed.
CONCLUSIONS
Anti-SAE DM represents a disease subset characterised by classic cutaneous involvement often associated with itching, less severe muscle involvement, but potential pulmonary involvement that should always be investigated in these patients.
Topics: Humans; Dermatomyositis; Autoantibodies; Myositis; Pruritus; Italy
PubMed: 38488098
DOI: 10.55563/clinexprheumatol/110r0p -
Clinical and Experimental Rheumatology Feb 2024To investigate the prevalence and characteristics of typical polymyositis (PM) in Chinese patients with idiopathic inflammatory myopathy (IIM).
OBJECTIVES
To investigate the prevalence and characteristics of typical polymyositis (PM) in Chinese patients with idiopathic inflammatory myopathy (IIM).
METHODS
Patients diagnosed with IIM according to the 2017 EULAR/ACR criteria were included. Serological aspects including myositis-specific antibodies (MSA) and pathological data were re-evaluated. The diagnosis of typical PM was strictly done using the pathological criteria, while excluding other IIM subtypes such as dermatomyositis (DM), immune-mediated necrotising myopathies (IMNM), anti-synthetase syndrome (ASS), and sporadic inclusion body myositis (sIBM), based on their respective diagnostic criteria.
RESULTS
A total of 544 IIM patients with muscle biopsy were involved, and 129 of them were diagnosed with initial PM according to the 2017 EULAR/ACR criteria. Only 6 (1.1%, 6/544) patients met the strict definition of typical PM after re-evaluation. Patients with typical PM were MSA-negative (100% vs. 35.7%, p=0.003) and had CD8+ T cells surrounding or invading non-necrotic muscle fibres in muscle biopsies (100% vs. 7.8%, p<0.001) compared to the initially diagnosed PM patients. All typical PM patients achieved clinical remission at the second-year follow-up. Typical PM patients had a favourable prognosis compared to MSA-negative IMNM and unspecific myositis patients.
CONCLUSIONS
Strictly defined typical PM is a rare clinical subtype in Chinese IIM patients. Typical PM patients with classical pathology were MSA-negative and responded well to treatment and had a favourable prognosis. It is crucial for clinicians to combine clinical, serological, and pathological features to properly distinguish PM from other IIM subtypes.
Topics: Humans; Myositis; Polymyositis; Myositis, Inclusion Body; Antibodies; Autoimmune Diseases; China; Autoantibodies
PubMed: 38488095
DOI: 10.55563/clinexprheumatol/7v9d2x -
Clinical and Experimental Rheumatology Feb 2024Psychological and emotional well-being are critical aspects of overall health for individuals with chronic rheumatologic conditions. Mental health-related literature,... (Review)
Review
Mental health in paediatric and adult myositis-related diseases: current state of research, interventions, and future steps from the MIHRA Psychological Impact Scientific Working Group.
Psychological and emotional well-being are critical aspects of overall health for individuals with chronic rheumatologic conditions. Mental health-related literature, however, predominantly focuses on systemic lupus erythematosus or rheumatoid arthritis, with limited emphasis on idiopathic inflammatory myopathies (IIMs). High proportions of those with juvenile myositis report psychological distress at levels warranting mental health referral. Adults with dermatomyositis diagnosed with depression or anxiety do not receive adequate mental health care. Mental health symptoms in those with IIMs are associated with worse health-related quality of life, medication adherence, and disease outcomes. Despite demonstrated high rates of mental health burden, access to mental health care remains severely lacking.Data related to mental health burden is limited by small sample size, limited generalisability, variable methods of assessment, and inconsistent diagnosis codes to define mental health conditions. Additional research is needed to validate current screening tools in myositis populations. Other relevant measurable factors include disease severity, non-health- and health-related trauma exposure, loneliness, isolation, loss of control, sleep difficulties, fatigue, pain, self-esteem, body image, sexual health, and health inequities. Studiesare needed investigating the efficacy of therapeutic and pharmacologic interventions among patients with myositis who experience depression and anxiety. Currently, knowledge and resources are limited around mental health burden and potential intervention for those living with IIMs. The Myositis International Health & Research Collaborative Alliance (MIHRA) Psychological Impact Scientific Working Group offers a preliminary road map to characterise and prioritise the work ahead to understand baseline mental health burden and compare avenues for intervention.
Topics: Adult; Humans; Child; Mental Health; Quality of Life; Global Health; Myositis; Dermatomyositis
PubMed: 38488093
DOI: 10.55563/clinexprheumatol/cngdfn -
Clinical and Experimental Rheumatology Feb 2024To characterise clinical amyopathic dermatomyositis (CADM) from a clinical, histological, and prognostic perspective.
OBJECTIVES
To characterise clinical amyopathic dermatomyositis (CADM) from a clinical, histological, and prognostic perspective.
METHODS
We retrospectively recorded data from our DM cohort. Patients were categorised into three groups: classic DM, hypomyopathic DM (HDM), characterised by normal muscle strength and evidence of muscle involvement in laboratory tests and/or instrumental examinations and CADM, featured by normal muscle strength and unremarkable findings in both laboratory tests and instrumental examinations. Available muscle biopsies from each group were also compared.
RESULTS
Our cohort included 63 DM (69.2%), 12 HDM (13.2%) and 16 CADM (17.6%) patients. Compared to DM, CADM patients were younger at onset and diagnosis (45.5±17 vs. 57±18, and 46±17 vs. 58±18 years, respectively; p<0.05). They were more likely to test positive for anti-MDA5 (37.5% vs. 4.8%) and anti- TIF1-γ (31.3% vs. 6.3%), had a higher incidence of arthritis (37.5% vs. 12.6%) and interstitial lung disease (ILD) (43.8% vs. 15.9%) (all comparisons with p<0.05). Muscle biopsies were available for 44 DM, 7 CADM, and 11 HDM patients, revealing similar sarcolemma MHC-I expression rates. Five-year survival rates were comparable across groups (DM: 74.6%, CADM: 75%, HDM: 83.3%). Cox analysis indicated the main mortality predictors in overall cohort were ILD (HR: 3.57, CI: 1.11-11.5) and cancer (HR: 3.67, CI: 1.17-11.5), not CADM (HR: 1.46, CI: 0.33-6.68).
CONCLUSIONS
CADM patients differ in disease onset, autoantibody profiles, joint and lung involvement. While laboratory and instrumental tests have not shown muscle involvement in CADM, many muscle biopsies have shown MHC-I overexpression.
Topics: Humans; Prognosis; Retrospective Studies; Dermatomyositis; Lung Diseases, Interstitial; Autoantibodies; Interferon-Induced Helicase, IFIH1
PubMed: 38488091
DOI: 10.55563/clinexprheumatol/kgpnbq -
Diagnostics (Basel, Switzerland) Feb 2024This study evaluates the antibody responses to SARS-CoV-2 vaccines in patients with neuroimmunological disorders (pwNID) who are receiving immunomodulating treatments,...
This study evaluates the antibody responses to SARS-CoV-2 vaccines in patients with neuroimmunological disorders (pwNID) who are receiving immunomodulating treatments, compared to healthy individuals. It included 25 pwNID with conditions such as optic neuritis, neuromyelitis optica spectrum disorder, multiple sclerosis, myasthenia gravis, and polymyositis, as well as 56 healthy controls. All participants had completed their full SARS-CoV-2 vaccination schedule, and their blood samples were collected within six months of their last dose. The concentration of anti-SARS-CoV-2 IgG antibodies was measured using an enzyme-linked immunosorbent assay. The results showed that pwNID had significantly lower antibody titers (58.4 ± 49.2 RU/mL) compared to healthy individuals (81.7 ± 47.3 RU/mL). This disparity persisted even after adjusting for age and the interval between the final vaccination and sample collection. A notable correlation was found between the use of immunomodulating treatments and reduced antibody levels, whereas mRNA vaccines were linked to higher antibody concentrations. The conclusion of this study is that immunomodulating treatments may reduce the effectiveness of SARS-CoV-2 vaccines in pwNID. This insight is crucial for healthcare providers in designing vaccination strategies and managing treatment plans for pwNID on immunomodulating therapies, highlighting the need for personalized approaches in this subgroup.
PubMed: 38472974
DOI: 10.3390/diagnostics14050502 -
Scientific Reports Mar 2024Little is known about a possible association of autoimmune inner ear disease among patients diagnosed with polymyositis (PM)/dermatomyositis (DM). This study aimed to...
Little is known about a possible association of autoimmune inner ear disease among patients diagnosed with polymyositis (PM)/dermatomyositis (DM). This study aimed to explore differences in the prevalence of inner ear symptoms among patients with and without PM/DM using a nationwide population-based dataset. Data for this study were retrieved from the Taiwan National Health Insurance Research Database. The study sample included 1622 patients diagnosed with PM/DM and 8109 propensity-score matched comparison patients without PM/DM. We performed multivariate logistic regressions to calculate odds ratios (ORs) and 95% confidence interval (CI) for tinnitus, hearing loss, sudden deafness, and vertigo among patients with PM/DM versus comparison patients. Chi-square tests showed statistically significant differences between patients with PM/DM and comparison patients in the prevalence of tinnitus (16.1% vs. 12.7%, p < 0.001), non-conductive hearing loss (9.2% vs. 6.8%, p < 0.001), and vertigo (14.4% vs. 11.1%, p < 0.001). The adjusted ORs for tinnitus, non-conductive hearing loss, and vertigo, respectively, were 1.332 (95% CI = 1.147-1.547), 1.399 (95% CI = 1.154-1.696), and 1.374 (95% CI = 1.173-1.611) for patients with PM/DM when compared to comparison patients. Our study finds that patients with PM/DM have higher prevalence rates of tinnitus, non-conductive hearing loss, and vertigo than comparison patients.
Topics: Humans; Animals; Dermatomyositis; Hearing Loss, Sudden; Tinnitus; Prevalence; Polymyositis; Deafness; Vertigo; Gastropoda
PubMed: 38459190
DOI: 10.1038/s41598-024-56372-z