- Elevated serum YKL-40 correlates with clinical characteristics in patients with polymyositis or dermatomyositis. [Journal Article]
- ACAnn Clin Biochem 2018 Jan 01; :4563218786979
- Background Serum YKL-40 has been proved to be a promising biomarker for estimating the disease activity of several autoimmune diseases. However, its utility in polymyositis or dermatomyositis has not...
Background Serum YKL-40 has been proved to be a promising biomarker for estimating the disease activity of several autoimmune diseases. However, its utility in polymyositis or dermatomyositis has not been established. The aim of this study was to investigate the utility of YKL-40 in patients with polymyositis/dermatomyositis. Method Patients with definite polymyositis/dermatomyositis who visited the Second People's Hospital of Wuxi between April 2016 and March 2017 were prospectively enrolled. Eighty-seven healthy individuals were set as a control. Serum YKL-40 of all participants was determined using ELISA. The associations between YKL-40 and clinical characteristics of polymyositis/dermatomyositis were analysed using the Student's t-test, Mann-Whitney test and receiver operating characteristic curve analysis. Results A total of 99 patients with polymyositis/dermatomyositis were enrolled. The patients with polymyositis/dermatomyositis had significantly higher serum YKL-40 concentration. Patients with interstitial lung disease had significantly higher YKL-40 concentration than those without. Serum YKL-40 was positively correlated with myositis disease activity assessment visual analogue scale, C-reactive protein, erythrocyte sedimentation rate and ferritin. The area under receiver operating characteristic curve of YKL-40 for identifying interstitial lung disease was 0.82. Conclusions Serum YKL-40 is a useful biomarker for estimating disease activity or severity of polymyositis/dermatomyositis.
- Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis. [Journal Article]
- RRheumatology (Oxford) 2018 Jul 16
- CONCLUSIONS: CAJDM may be distinguished from JDM, in that they often have p155/140 (transcriptional intermediary factor 1) autoantibodies, have fewer systemic manifestations and receive less therapy.
- Anncaliia algerae Microsporidial Myositis, New South Wales, Australia. [Journal Article]
- EIEmerg Infect Dis 2018; 24(8):1528-1531
- We describe the successful management of Anncaliia algerae microsporidial myositis in a man with graft versus host disease after hemopoietic stem cell transplantation. We also summarize clinical pres...
We describe the successful management of Anncaliia algerae microsporidial myositis in a man with graft versus host disease after hemopoietic stem cell transplantation. We also summarize clinical presentation and management approaches and discuss the importance of research into the acquisition of this infection and strategies for prevention.
- A Young Male with Severe Myocarditis and Skeletal Muscle Myositis. [Journal Article]
- CRCase Rep Cardiol 2018; 2018:5698739
- A 34-year-old male presented with retrosternal chest pain, fatigue, shortness of breath, and a history of a previous episode of myocarditis four years prior. He had elevated troponin T, normal skelet...
A 34-year-old male presented with retrosternal chest pain, fatigue, shortness of breath, and a history of a previous episode of myocarditis four years prior. He had elevated troponin T, normal skeletal muscle enzymes, and negative inflammatory markers. Cardiac magnetic resonance imaging (MRI) confirmed active myocarditis with extensive myocardial fibrosis and normal left ventricular ejection fraction (LVEF). His myocarditis symptoms resolved with steroids and anti-inflammatory treatment, but on closer questioning, he reported a vague history of long-standing calf discomfort associated with episodes of stiffness, fatigue, and flu-like symptoms. MRI of the lower legs consequently demonstrated active myositis in the calf muscles. Immunomodulatory therapy was commenced with good effect. The patient is undergoing regular follow-up in both cardiology and rheumatology outpatient departments. Repeated MRI of the legs showed significant interval improvement in his skeletal muscle myositis, and repeat cardiac MRI demonstrated the resolution of myocarditis along with persistent stable extensive myocardial fibrosis and preserved LVEF. The patient has returned to full-time work.
- Real-Life Evidence for Tedizolid Phosphate in the Treatment of Cellulitis and Wound Infections: A Case Series. [Journal Article]
- IDInfect Dis Ther 2018 Jul 12
- CONCLUSIONS: Tedizolid phosphate 200 mg for 7-14 days was a favored treatment option for patients with severe/complex ABSSSIs, and was effective following previous treatment failure or in late-onset infections.
- Sensitivity and clinical utility of the anti-cytosolic 5'-nucleotidase 1A (cN1A) antibody test in sporadic inclusion body myositis: Report of 40 patients from a single neuromuscular center. [Journal Article]
- NDNeuromuscul Disord 2018 Jun 18
- Sporadic inclusion body myositis (IBM) is the most common acquired myopathy affecting patients over age 50. The discovery of an autoantibody directed against a 43-44 kD protein (anti-cytosolic-5'-nuc...
Sporadic inclusion body myositis (IBM) is the most common acquired myopathy affecting patients over age 50. The discovery of an autoantibody directed against a 43-44 kD protein (anti-cytosolic-5'-nucleotidase 1A or anti-cN1A) has provided support for the hypothesis of an immune-mediated pathogenesis. Previous studies have reported variable test sensitivity and specificity, and inconsistent results on the predictive value. In our cohort of 40 patients with clinico-pathologically or clinically defined IBM, we found the sensitivity of the anti-cN1A antibody test to be 50%. Comparing characteristics for test positive and test negative groups, we found that patients in our cohort testing positive for the anti-cN1A antibody were significantly more likely to be older than age 60 years at symptom onset. We found no positive association between anti-cN1A reactivity and other clinical, laboratory, and muscle histopathologic findings. Based on all clinical studies published to date including the present, the anti-cN1A antibody test shows high diagnostic specificity, moderate sensitivity, and a low predictive value in regards to age of onset, disease severity and other associated clinicopathological findings.
- The Diagnostic Value of MRI Pattern Recognition in Distal Myopathies. [Journal Article]
- FNFront Neurol 2018; 9:456
- Objective: Distal myopathies are a diagnostically challenging group of diseases. We wanted to understand the value of MRI in the current clinical setting and explore the potential for optimizing its...
Objective: Distal myopathies are a diagnostically challenging group of diseases. We wanted to understand the value of MRI in the current clinical setting and explore the potential for optimizing its clinical application. Methods: We retrospectively audited the diagnostic workup in a distal myopathy patient cohort, reassessing the diagnosis, whilst documenting the usage of MRI. We established a literature based distal myopathies MRI pattern template and assessed its diagnostic utility in terms of sensitivity, specificity, and potential impact on the diagnostic workup. Results: Fifty-five patients were included; in 38 with a comprehensive set of data the diagnostic work-up was audited. The median time from symptoms onset to diagnosis was 12.1 years. The initial genetic diagnostic rate was 39%; 18% were misdiagnosed as neuropathies and 13% as inclusion body myositis (IBM). Based on 21 publications we established a MRI pattern template. Its overall sensitivity (50%) and specificity (32%) were low. However in some diseases (e.g., MYOT-related myopathy, TTN-HMERF) MRI correctly identified the causative gene. The number of genes suggested by MRI pattern analysis was smaller compared to clinical work up (median 1 vs. 9, p < 0.0001) but fewer genes were correctly predicted (5/10 vs. 7/10). MRI analysis ruled out IBM in all cases. Conclusion: In the diagnostic work-up of distal myopathies, MRI is useful in assisting genetic testing and avoiding misdiagnosis (IBM). The overall low sensitivity and specificity limits its generalized use when traditional single gene test methods are applied. However, in the context of next generation sequencing MRI may represent a valuable tool for interpreting complex genetic results.
- [Myositis ossificans progressia of lateral pterygoid muscle of both sides: a case report]. [Journal Article]
- ZKZhonghua Kou Qiang Yi Xue Za Zhi 2018 Jul 09; 53(7):488-489
- Outcome measures of disease activity for rare autoimmune rheumatic diseases. [Journal Article]
- BJBr J Hosp Med (Lond) 2018 Jul 02; 79(7):396-401
- Systemic lupus erythematosus, scleroderma, myositis and Sjögren's syndrome are rare, complex, multi-systemic rheumatic diseases associated with significant morbidity and mortality. Thorough assessmen...
Systemic lupus erythematosus, scleroderma, myositis and Sjögren's syndrome are rare, complex, multi-systemic rheumatic diseases associated with significant morbidity and mortality. Thorough assessments of disease activity are required to guide clinical management and assess response to new therapies in clinical trials. This article reviews the commonly used outcome measures to assess this group of diseases and discusses the limitations of their use.
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- CD19+CD24hiCD38hi B Cells Are Expanded in Juvenile Dermatomyositis and Exhibit a Pro-Inflammatory Phenotype After Activation Through Toll-Like Receptor 7 and Interferon-α. [Journal Article]
- FIFront Immunol 2018; 9:1372
- Juvenile dermatomyositis (JDM) is a rare form of childhood autoimmune myositis that presents with proximal muscle weakness and skin rash. B cells are strongly implicated in the pathogenesis of the di...
Juvenile dermatomyositis (JDM) is a rare form of childhood autoimmune myositis that presents with proximal muscle weakness and skin rash. B cells are strongly implicated in the pathogenesis of the disease, but the underlying mechanisms are unknown. Therefore, the main objective of our study was to investigate mechanisms driving B cell lymphocytosis and define pathological features of B cells in JDM patients. Patients were recruited through the UK JDM Cohort and Biomarker study. Peripheral blood B cell subpopulations were immunophenotyped by flow cytometry. The results identified that immature transitional B cells were significantly expanded in active JDM, actively dividing, and correlated positively with disease activity. Protein and RNAseq analysis revealed high interferon alpha (IFNα) and TLR7-pathway signatures pre-treatment. Stimulation of B cells through TLR7/8 promoted both IL-10 and IL-6 production in controls but failed to induce IL-10 in JDM patient cells. Interrogation of the CD40-CD40L pathway (known to induce B cell IL-10 and IL-6) revealed similar expression of IL-10 and IL-6 in B cells cultured with CD40L from both JDM patients and controls. In conclusion, JDM patients with active disease have a significantly expanded immature transitional B cell population which correlated with the type I IFN signature. Activation through TLR7 and IFNα may drive the expansion of immature transitional B cells in JDM and skew the cells toward a pro-inflammatory phenotype.