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BACKGROUND: Myositis is a rare disease associated with impaired health-related quality of life. A study evaluating the effectiveness of an intervention to improve the quality of life and well-being of myositis patients is presented. METHODS: All myositis patients in a health district were contacted. Thirty-four eligible patients were randomly assigned to the experimental (n = 17) or control (n = 17) group. A psychoeducational intervention of 5 100-min sessions focusing on the disease as related to daily life was conducted only in experimental patients. Several reliable tools to measure quality of life and well-being were administered twice, before and after the intervention, to both groups. RESULTS: In the experimental group, post-test scores were higher than pre-test in quality of life, well-being, and self-efficacy to manage the disease. Improvements were more evident in the experimental group than controls in 70% of the variables studied. Specifically, sedentariness decreased and satisfaction with social relationships increased in the post-test evaluation to a greater degree in the experimental group than in controls. CONCLUSIONS: This randomized controlled trial on a representative sample of myositis patients in an extensive population provides evidence indicating the effectiveness of a psychoeducational intervention for improving HRQoL, well-being, and self-efficacy to manage the disease. TRIAL REGISTRATION: NCT06300983.
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Miosite , Qualidade de Vida , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , IdosoRESUMO
BACKGROUND: Anti-mitochondrial antibody (AMA)-positive myositis is a chronic disease characterized by skeletal muscle atrophy and is associated with cardiac complications and restrictive ventilatory impairment. This study aimed to determine the prevalence, rate of organ complications, and prognostic risk factors of AMA-positive myositis. METHODS: We conducted a cross-sectional study using a nationwide questionnaire from 2011 to 2021, enrolling participants from neurology departments at 811 facilities certified by the Japanese Society of Neurology. RESULTS: A total of 380 patients were identified, with a prevalence rate of 0.3 per 100,000 persons. The frequencies of cardiac complications and restrictive ventilatory impairment were 53 and 33 %, respectively; whereas, those of cardiac device and respirator introduction were 32 and 22 %, respectively. The frequencies of recurrence, subacute exacerbation, no muscle strength improvement, cardiac device introduction, respirator introduction, and death were 29, 25, 54, 32, 22, and 12 %, respectively. According to univariate analysis, abnormal echocardiograms (odds ratio [OR], 5.43), restrictive ventilatory impairment (OR, 3.70), and inflammatory changes revealed by muscle biopsy (OR, 0.34) were associated with subacute exacerbations, whereas abnormal echocardiograms (OR, 8.00) and durations from onset to admission and diagnosis (OR, 2.99) were associated with cardiac device introduction. Multivariable analysis showed that restrictive ventilatory impairment was associated with recurrence (adjusted OR, 3.01), adjusted for the duration from onset to admission and diagnosis, and with subacute exacerbations (adjusted OR, 3.86), adjusted for abnormal echocardiograms and inflammatory changes. CONCLUSIONS: AMA-positive myositis is characterized by severe and urgent organ complications, and anticipatory management is critical for management of this disease.
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Immunotherapy is increasingly used in advanced non-small-cell lung cancer (NSCLC), offering a significant anti-tumor response, as well as causing rising immune-related adverse effects. The incidence of immune checkpoint inhibitor-induced myocarditis-myositis-myasthenia gravis is increasing and particularly concerning due to its high mortality rate. Prompt recognition, diagnosis, and management are crucial. A 40-year-old patient, diagnosed with stage IV non-oncogene addicted lung adenocarcinoma, with nivolumab-ipilimumab-chemotherapy as first-line treatment, developed a rare myocarditis-myositis-myasthenia gravis overlap syndrome. Following the treatment, the patient presented with flu-like symptoms and chest pain and subsequently transferred to the cardiac intensive care unit. The physical examination revealed a visual acuity deficit, diplopia, ophthalmoparesis, ptosis, mydriasis, dysphagia, dyspnea, headache, nausea, dry mouth, asthenia, myalgia, and muscle weakness. Imaging and laboratory tests confirmed the triad, showing an elevation of hs-cTnI and CK and positive results for anti-SAE1 and anti-PL-7 Abs. ECG revealed ST segment elevation and RBBB. The echo showed hyperechogenicity of the inferolateral wall, pericardial detachment, and thickening. The cardiac MRI demonstrated hypokinesia, edema, subepicardial LGE, and pericardial effusion. Muscle biopsy revealed muscle fiber necrosis and regeneration with B and T lymphocytic endomysial inflammatory infiltrate and expression of MHC-I. Treatment with oral prednisone, pyridostigmine, and IV Igs was started due to poor clinical response followed by methylprednisolone. Despite stopping immunotherapy, the patient continued to benefit from it, as highlighted on subsequent re-evaluation CT scans by partial disease response, and as the patient was in complete remission, we decided to resume chemotherapy by omitting immunotherapy. At the radiological control following the four cycles of double CHT and during CHT maintenance, there was a further reduction of the disease. This report aims to raise awareness among physicians about these serious side effects. A multidisciplinary approach led to clinical improvement and early intervention, optimizing patient outcomes.
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Sporadic inclusion body myositis (sIBM), the most common inflammatory muscle disorder in adults over 50 years, is often misdiagnosed due to its gradual onset and its common but unspecific muscle weakness in older adults. Diagnosis relies on clinical, radiological, and pathological features. Cardiac involvement is rare, prompting this case description and a comprehensive literature analysis. A 73-year-old woman diagnosed with sIBM in 2021 through muscle biopsy had been experiencing muscular symptoms since 2015. Her condition progressively worsened, affecting daily activities. Annual follow-ups revealed a moderate obstructive syndrome on respiratory testing, prompting a cardiac evaluation. Cardiac magnetic resonance (CMR) imaging identified intramyocardial lesions consistent with fatty infiltration, highlighting the interest of advanced imaging in sIBM management. Cardiac involvement in sIBM is presumed rare compared to other idiopathic inflammatory myopathies, though the exact frequency remains unclear. Early identification of heart alterations by CMR in sIBM can be prognostically valuable, guiding follow-up and interventions. However, literature on this subject is limited to small cohort studies and case reports describing complications. Given the slow progression of sIBM and the limited efficacy of current treatments, the discovery of myocardial lesions could warrant closer cardiological monitoring. Larger cohort studies are needed to explore potential new therapeutic approaches. Our case underscores the importance of CMR in detecting subtle cardiac manifestations in sIBM and illustrates the potential prognostic value of cardiac assessment in the management of sIBM.
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This review highlights the key role of imaging modalities in diagnosing and managing myositis. The authors underscore MRI's superiority in identifying muscle edema and fat infiltration, marking it as essential for evaluating disease activity and damage. They also suggest ultrasound's emerging significance for diagnosis and monitoring of the disease, valued for its ease of use, and real-time capabilities. Furthermore, PET scans' unique physiologic capabilities, especially useful for malignancy detection and assessing lung disease, are emphasized. Collectively, these imaging techniques offer a tailored approach to myositis management, facilitate precise diagnosis, effective treatment planning, and disease activity monitoring, thereby enhancing patient outcomes in rheumatology practice.
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Imageamento por Ressonância Magnética , Miosite , Tomografia por Emissão de Pósitrons , Ultrassonografia , Humanos , Miosite/diagnóstico , Miosite/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Ultrassonografia/métodos , Tomografia por Emissão de Pósitrons/métodos , Músculo Esquelético/diagnóstico por imagem , Músculo Esquelético/patologia , Tomografia Computadorizada por Raios XRESUMO
An adult, female, mixed breed sow from a commercial breeding unit, with a clinical history of lethargy, poor body condition, hypersalivation, dyspnoea and reddened ears, was submitted for necropsy. Post-mortem examination identified the presence of an intestinal volvulus, which developed around an osseous intra-abdominal mesenteric lesion. Gross pathology and histopathology combined with post-mortem computed tomography (CT) of the mass confirmed the lesion as intra-abdominal heterotopic mesenteric ossification (IHMO), according to criteria applied to human disease. This report highlights a rare case of porcine IHMO, a poorly understood metaplastic condition, comparable to the human disease. We highlight the importance of a combination of CT and histopathology to reach a final diagnosis of the disease. Histopathology and further investigation of similar cases may contribute to a better understanding of the pathogenesis in animals, which is currently poorly understood.
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Herpes zoster ophthalmicus results from the reactivation of the latent varicella zoster virus, affecting the first branch of the trigeminal nerve. In 20-70% of cases, Zoster Ophthalmicus can lead to ocular involvement, affecting various orbital structures. Orbital myositis is a rare but severe complication of herpes zoster ophthalmicus. We present a case of a 52-year-old man with no significant medical history who developed zoster-associated right ocular myositis and dacryocystitis. He was treated with intravenous acyclovir and oral steroids. A review of the literature identified 29 patients across 19 studies. The median age was 61 years, with a slight female predominance. In 55% of cases, the patients had no notable medical history. The most common presentation of myositis involved all oculomotor muscles. There were 22 cases who were treated with intravenous antiviral therapy and 19 received steroids. A full resolution of symptoms was achieved in 51.7% of patients. Zoster-related orbital myositis is a rare complication that should be considered even in immunocompetent individuals. It may occur either before or after the appearance of a vesicular rash. Magnetic resonance imaging is the preferred radiological exam for assessing orbital involvement. Intravenous antiviral therapy should be started within 72 h of symptom onset, and its combination with systemic corticosteroids appears to be an effective treatment for zoster-related ocular myositis.
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Th17 cells are known for producing IL-17 and their role in the pathogenesis of various autoimmune diseases, including myositis. Likewise, the participation of the IL-23/IL-17 pathway in autoimmunity has been confirmed. In this study, we aimed to evaluate the behavior of cytokines in myositis, focusing on the autoantibodies profile and the myositis core set measures. Twenty-five myositis patients were enrolled in this cross-sectional study. An expert rheumatologist evaluated the myositis core set measures. Serum levels of cytokines and chemokines were quantified using the LEGENDplex Multi-Analyte Flow Assay Kit from BioLegend. The autoantibodies detection was carried out using the line-blot assay kit Euroline: Autoimmune Inflammatory Myopathies from EUROIMMUN. We found higher serum levels of IL-33, CXCL8, IL-6, IL-23, and IL-12p70 in seronegative patients. A multiple linear regression analysis revealed that MYOACT scores could be predicted by the increment of IL-23 and the decrement of CCL2, IL-10, and CXCL8 serum levels. These findings suggest that the immune response in seronegative myositis patients exhibits an IL-23-driven Th17 immune response. The relevance of this discovery lies in its potential therapeutic implications. Insights into the IL-23-driven Th17 immune response in seronegative patients highlight the potential for targeted therapies aimed at modulating Th17 activity.
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Citocinas , Miosite , Células Th17 , Humanos , Miosite/imunologia , Miosite/sangue , Feminino , Masculino , Células Th17/imunologia , Células Th17/metabolismo , Pessoa de Meia-Idade , Citocinas/sangue , Adulto , Estudos Transversais , Autoanticorpos/sangue , Autoanticorpos/imunologia , Interleucina-23/sangue , IdosoRESUMO
Fibrosing interstitial lung diseases (FILDs) other than idiopathic pulmonary fibrosis (IPF) can develop into progressive pulmonary fibrosis (PPF) despite initial management. A substantial proportion of patients with non-IPF interstitial lung diseases (ILDs) progress to PPF, including connective tissue disease-associated ILD (such as rheumatoid arthritis-associated ILD, systemic sclerosis-associated ILD, and idiopathic inflammatory myositis-associated ILD), fibrosing hypersensitivity pneumonitis, and fibrosing occupational ILD. The concept of PPF emerged only recently and several studies have confirmed the impact of PPF on mortality. In addition to poor prognosis among patients with PPF, there remains a lack of consensus in the diagnosis and treatment of PPF across different types of ILDs. There is a need to raise awareness of PPF in FILDs and to explore measures to improve PPF diagnosis and treatment, which in turn could potentially reduce the progression from FILD to PPF. This review discusses the disease burden of PPF and recent advances in the management of PPF among patients with ILDs, including antifibrotic medications that have emerged as promising treatment options. Additionally, this review highlights the perspectives of expert Chinese physicians with regard to their experience in managing PPF in clinical practice.
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Progressão da Doença , Fibrose Pulmonar , Humanos , Antifibróticos/uso terapêutico , China , Pulmão/fisiopatologia , Pulmão/patologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/terapia , Doenças Pulmonares Intersticiais/fisiopatologia , Doenças Pulmonares Intersticiais/etiologia , Fibrose Pulmonar/diagnóstico , Fibrose Pulmonar/terapia , Fibrose Pulmonar/fisiopatologia , Resultado do TratamentoRESUMO
BACKGROUND: Post-streptococcal myalgia and myositis are very rare complications of streptococcal infections with group A ß-haemolytic streptococci. Data on this condition are scarce and even less is known about findings in clinical imaging. Until today, there are no descriptions of ultrasonographic changes in this condition. CASE PRESENTATION: We present a case of a 31-year-old female patient with immobilizing myalgia of the left outer thigh following a streptococcal upper respiratory tract infection, accompanied with erythemata nodosa on both shins. Laboratory results indicated post-streptococcal myositis since Creatine kinase, Lactate dehydrogenase and Antistreptolysin antibodies were significantly elevated. An ultrasound of the affected vastus medialis of the left quadriceps femoris muscle was performed, which showed a focal increase in muscle echogenicity with loss of architecture and hypervascularisation in Power Doppler Mode. The diagnosis of focal myositis was confirmed with magnetic resonance imaging. The patient's symptoms as well as the ultrasonographic changes fully resolved under therapy with Ibuprofen and intravenous Ampicillin/Sulbactam. CONCLUSIONS: This is the first description of ultrasound findings in this rare condition. We conclude that muscular ultrasound is helpful to identify myositis in post-streptococcal myalgia and myositis.
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Miosite , Infecções Estreptocócicas , Humanos , Feminino , Adulto , Miosite/diagnóstico por imagem , Miosite/microbiologia , Miosite/tratamento farmacológico , Infecções Estreptocócicas/diagnóstico por imagem , Infecções Estreptocócicas/microbiologia , Infecções Estreptocócicas/tratamento farmacológico , Imageamento por Ressonância Magnética , Antibacterianos/uso terapêutico , Mialgia/etiologia , Mialgia/microbiologia , Mialgia/diagnóstico por imagem , Músculo Quadríceps/diagnóstico por imagem , Músculo Quadríceps/microbiologia , UltrassonografiaRESUMO
Statins are widely prescribed to patients for their efficiency in cholesterol management. However, they carry the risk of inducing a relatively rare, serious, and potentially life-threatening condition known as statin-induced necrotizing autoimmune myopathy (SINAM). This case report provides an in-depth analysis of SINAM, focusing on its epidemiology, clinical presentation, and the challenges involved in accurate diagnosis. In addition, we review evolving theories addressing its pathogenesis and current therapeutic approaches. This report identifies the need for early diagnosis and intervention and recommends a multidisciplinary approach to optimize patient outcomes. This article also highlights the need for further research studies to enhance our understanding of SINAM and its management.
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The overexpression of major histocompatibility complex (MHC) I on the surface of muscle fibers is a characteristic hallmark of the idiopathic inflammatory myopathies (IIMs), collectively termed myositis. Alongside MHC-I overexpression, subtypes of myositis, display a distinct type I interferon (IFN) signature. This study examined the combinational effects of elevated MHC-I and type I IFNs (IFNα/ß) on mitochondrial function, as mitochondrial dysfunction is often seen in IIMs. Human skeletal muscle myoblasts were transfected with an MHC-I isoform using the mammalian HLA-A2/Kb vector. Mitochondrial respiration, mitochondrial membrane potential, and reactive oxygen/nitrogen species generation were assessed with or without IFNα and IFNß. We show that MHC-I overexpression in human skeletal muscle myoblasts led to decreased basal glycolysis and mitochondrial respiration, cellular spare respiratory capacity, adenosine triphosphate-linked respiration, and an increased proton leak, which were all exaggerated by type I IFNs. Mitochondrial membrane depolarization was induced by MHC-I overexpression both in absence and presence of type I IFNs. Human myoblasts overexpressing MHC-I showed elevated nitric oxide generation that was abolished when combined with IFN. MHC-I on its own did not result in an increased reactive oxygen species (ROS) production, but IFN on their own, or combined with MHC-I overexpression did induce elevated ROS generation. Surprisingly, we observed no gross changes in mitochondrial reticular structure or markers of mitochondrial dynamics. We present new evidence that MHC-I overexpression and type I IFNs aggravate the effects each has on mitochondrial function in human skeletal muscle cells, providing novel insights into their mechanisms of action and suggesting important implications in the further study of myositis pathogenesis.
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BACKGROUND: Patients with idiopathic interstitial pneumonia (IIP) often exhibit positivity for myositis-specific antibodies (MSA). However, the significance of this finding remains unclear. In this study, we investigated the association of MSA with the prognosis and risk of acute exacerbation in patients with IIP. METHODS: We retrospectively reviewed the medical records of patients with IIP and examined the effect of each MSA subtype on survival and acute exacerbation. RESULTS: Of 240 patients with IIP, 48 (20%) exhibited positivity for MSA. The MSA subtypes included: PL-7 (antithreonyl; n = 16, 6.7%); signal recognition particle (n = 13, 5.4%); PL-12 (antialanyl; n = 9, 3.8%); Mi-2 (n = 8, 3.3%); OJ (anti-isoleucyl; n = 7, 2.9%). During the 382 days (382 ± 281 days) of observation, 32 (13%) patients expired, and 27 (11%) experienced an acute exacerbation. Cox proportional hazards regression analysis demonstrated that age at the initial visit (hazard ratio [HR]: 1.072; 95% confidence interval [CI]: 1.017-1.131; P = 0.01), PL-7 (HR: 4.785; 95% CI: 1.528-14.925; P = 0.007), and PL-12 (HR: 3.922; 95% CI: 1.198-12.82; P = 0.024) were independent predictors of survival time. PL-7 (HR: 3.268; 95% CI: 1.064-10; P = 0.039) and PL-12 (HR: 5.747; 95% CI: 1.894-7.544; P = 0.002) were independent predictors of time from first visit to acute exacerbation. CONCLUSION: Detecting MSA in patients with interstitial lung disease may be useful in predicting prognosis and providing a rationale for intensive treatment.
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Autoanticorpos , Pneumonias Intersticiais Idiopáticas , Miosite , Humanos , Feminino , Masculino , Estudos Retrospectivos , Idoso , Prognóstico , Pessoa de Meia-Idade , Pneumonias Intersticiais Idiopáticas/mortalidade , Pneumonias Intersticiais Idiopáticas/diagnóstico , Pneumonias Intersticiais Idiopáticas/imunologia , Miosite/imunologia , Miosite/diagnóstico , Autoanticorpos/sangue , Progressão da Doença , Modelos de Riscos Proporcionais , Idoso de 80 Anos ou maisRESUMO
In recent years, various myositis-specific and myositis-associated autoantibodies have been identified in idiopathic inflammatory myopathies, including dermatomyositis (DM), anti-synthetase syndrome (ASS), immune-mediated necrotizing myopathy (IMNM), and inclusion body myositis (IBM). These autoantibodies exhibit unique characteristics in terms of organ involvement, severity, and treatment response, making their understanding crucial for accurate diagnosis and effective therapy. This review provides a comprehensive overview of the clinical features of recently discovered myositis-specific and associated autoantibodies, while exploring their potential roles in the pathogenesis and exacerbation of myositis. Key findings include the production of anti-TIF1γ antibodies in model mice, the upregulation of Mi2-related genes in anti-Mi2 antibody-positive dermatomyositis muscle tissue, and Jo-1 antigen-induced T cell activation, shedding light on whether disease mechanisms are driven by autoantibodies or autoantigens.
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Objectives: Focal myositis (FM) is a rare and restricted skeletal muscle inflammation, presenting as a solid mass with a typical lower leg localization and benign prognosis. In most cases the process solves spontaneously or after immunosuppressant therapy, but sometimes it recurs or progresses to a systemic inflammation. The basis of the disease are mostly unknown. Methods: Hence, we provide an update of histopathological features of FM, in order to better define the underlying pathomechanisms of this disorder. A PubMed literature search was focused on the case reports published in English from July 1977 to December 2023. Results: FM and other myositis may show similar morphological features. Emerging studies on MMP molecules and future eventual research on microRNAs (miRNAs) could help in differential diagnosis. Conclusions: Clinical, laboratory, neurophysiological and imaging findings can allow a correct diagnosis. However, muscle biopsy seems to be the only diagnostic tool to differentiate among FM and other localized soft tissue masses.
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Miosite , Humanos , Miosite/diagnóstico , Miosite/imunologia , Miosite/patologia , Diagnóstico Diferencial , Músculo Esquelético/patologiaRESUMO
Introduction: Profuse diarrhea and abdominal discomfort are well-documented symptoms of patients with known colorectal cancer. It is much less common for these patients to present with a chief complaint of gait disturbance and rhabdomyolysis. We present a case of incidentally discovered colorectal carcinoma in a patient who was initially evaluated for progressive weakness and recurrent falls. Case Presentation: A 51-year-old man was admitted to our department for management of rhabdomyolysis in the setting of progressive lower extremity weakness and mechanical falls. He developed abdominal discomfort and bowel changes during his admission, and after further investigation, he was found to have a rectal polyp positive for invasive adenocarcinoma, as well as multiple gluteal abscesses. Workup for metastasis, mutations, and oncogenic biomarkers was unremarkable. Conclusion: This case is a demonstration of a medically complex patient presentation compounded by multifactorial processes. Future providers may take note that an initial absence of classic gastrointestinal (GI) symptoms does not necessarily rule out underlying GI cancer. Instead, the initial presentation of colorectal adenocarcinoma may manifest with paraneoplastic versus incidental progressive proximal limb weakness prior to GI symptoms such as diarrhea. Additionally, our report demonstrates a case of possible paraneoplastic gluteal abscesses, which may have further contributed to the patient's gait disturbance. However, it is unclear as to whether our patient's various symptoms were directly linked to one another, or if they were incidental co-presentations.
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Inclusion body myositis is a idiopathic inflammatory myopathy characterized by muscle weakness and dysphagia, with muscle biopsy showing inflammation and rimmed vacuoles. We present the case of a patient who was diagnosed with polymyositis but due to lack of response to treatment, a new biopsy revealed inclusion body myositis.
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OBJECTIVES: To validate the 2018 European Neuromuscular Centre classification (ENMC) criteria, compare its performance to the 1975 Bohan & Peter (B&P) and 2017 European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) criteria for dermatomyositis (DM), and describe characteristics of different myositis-specific autoantibody (MSA)-positive patients defined by the ENMC-DM criteria. METHODS: Medical records and data on MSAs and muscle biopsies were retrospectively obtained from 1370 Chinese patients with idiopathic inflammatory myopathy (IIM) between 2008 and 2020. Patients were diagnosed with DM by at least two rheumatologists and classified according to the ENMC-DM, EULAR/ACR, and B&P criteria. RESULTS: Of the 1370 patients, 857, 671, 693, and 913 were diagnosed with DM using the specialists' gold standard, ENMC-DM, EULAR/ACR, and B&P criteria, respectively. Significant between-group differences were observed in the clinical symptoms, serum creatine kinase levels, and MSAs (P < 0.05). Based on muscle biopsy data, the B&P criteria had the highest sensitivity (94%) but lowest specificity (65%). Without muscle biopsy data, the ENMC-DM criteria had the highest specificity (92%) but lowest sensitivity (61%). The sensitivity and specificity of the EULAR/ACR criteria were intermediate (72% and 86%, respectively) regardless of muscle biopsy data availability. With MSA data, the sensitivity and specificity of the ENMC-DM criteria were 73% and 91% and increased to 76% and 97%, respectively, with both muscle biopsy and MSA data. CONCLUSIONS: The ENMC-DM criteria had higher specificity than the other criteria, especially in the absence of muscle biopsy data. Sensitivity and specificity improved when both muscle biopsy and MSA data were available. Key Points ⢠Idiopathic inflammatory myopathy presents diagnostic challenges due to its variable features and dermatomyositis has distinct subtypes based on myositis-specific autoantibodies (MSAs) with unique clinical phenotypes. ⢠This study validates the ENMC-DM criteria in Chinese patients and provides a comprehensive comparison with the B&P and EULAR/ACR criteria. ⢠It demonstrates that the new ENMC-DM criteria exhibit higher specificity, especially noteworthy in cases without muscle biopsy, and the study further highlights the improved sensitivity and specificity when combining muscle biopsy and MSAs, offering a refined approach for accurate DM classification.
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BACKGROUND: Eosinophilic esophagitis (EoE) presents with various esophageal motility disorders, and some cases of hypercontractile esophagus (HE) are associated with eosinophilic esophageal myositis (EoEM). This study aimed to compare the clinical characteristics of patients with EoE and EoEM according to their esophageal motility. METHODS: The 28 patients with EoE and 2 patients with EoEM were divided into three groups based on esophageal motility: normal motility group, hypomotility group, and spastic contraction group. The clinical characteristics of the three groups were retrospectively compared. RESULTS: Among the 28 patients with EoE, there were 15 with normal esophageal motility, 9 with hypomotility (2 with absent contractility, 7 with ineffective esophageal motility), and 4 with spastic contractions (1 with type III achalasia, 1 with HE, 2 with unclassifiable multipeak contractions). The two patients with EoEM had HE. Most patients in the normal and hypomotility groups had typical endoscopic findings of EoE, whereas these typical findings were less common in the spastic contraction group (P < 0.001). Four of the five patients with esophageal stricture were in the hypomotility group (P = 0.036). The therapy method significantly differed between the three groups: the normal group had more patients that responded to a proton pump inhibitor or potassium-competitive acid blocker, the hypomotility group had more patients that responded to steroids, and the spastic contraction group contained two patients treated with per-oral endoscopic myotomy (P = 0.021). CONCLUSIONS: The endoscopic findings and therapy methods differ between patients with EoE and EoEM based on the esophageal motility.