Background Juvenile dermatomyositis(JDM)is a chronic autoimmune disease characteristic by inflammation of small vessels within the skin,muscle and vital organs.But the clinical features and treatment of JDM have not b...Background Juvenile dermatomyositis(JDM)is a chronic autoimmune disease characteristic by inflammation of small vessels within the skin,muscle and vital organs.But the clinical features and treatment of JDM have not been fully clarified.Data sources Databases underwent through PubMed for articles about the clinical features,myositis-specific antibodiesof JDM and its treatment,and we selected publications written in English which were relevant to the topic of this review.Results Clinical features and myositis-specific antibodies may predict the severity and prognosis of disease.Although the mortality rate has been lower with traditional treatments,such as corticosteroid,intravenous immunoglobulin,and diseasemodifying anti-rheumatic drugs such as methotrexate,their usages are variable.Novel biological therapies seem to be effective for refractory JDM patients,but more clinical trials are necessary.Conclusions JDM is a sever disease of childhood.We need to better understand recent advances of JDM in the context of clinical features including skin manifestations,muscle weakness and organ damage,myositis-specific antibodies and their associated outcomes and the treatment of disease.展开更多
This study investigated the role of mast cells in canine cutaneous vascular tumors, and is the first such study to distinguish between tumors arising in the dermis versus the subcutis. Mast cell numbers in canine cuta...This study investigated the role of mast cells in canine cutaneous vascular tumors, and is the first such study to distinguish between tumors arising in the dermis versus the subcutis. Mast cell numbers in canine cutaneous hemangiomas (HA) and hemangiosarcomas (HSA) were evaluated to identify a relationship between mast cells, tumor type (HA, HSA), histologic location (dermis, subcutis) and tumor recurrence. One hundred and sixty-seven biopsies from 148 dogs were evaluated. Using only one biopsy from each dog, mast cell counts (MCC) for each tumor (n = 148) were obtained by averaging the number of mast cells counted in ten 400× fields. A significant difference in mean MCC was found only between tumor types, with HA having more mast cells than HSA (4.2 ± 4.2 vs. 2.2 ± 2.6;p < 0.001). No significant difference in mean MCC existed between tumors that recurred and those that did not. There was no difference in recurrence rate between tumor type or histologic location. Our results indicate that benign HA contain more mast cells than malignant endothelial cell tumors, regardless of histologic location;whether this is a cause or effect relationship remains to be determined.展开更多
Background: Response to rituximab so far is unpredictable in patients with refractory myositis. Predictive models of clinical improvement are developed using clinical, laboratory, and gene expression/cytokine/chemokin...Background: Response to rituximab so far is unpredictable in patients with refractory myositis. Predictive models of clinical improvement are developed using clinical, laboratory, and gene expression/cytokine/chemokine variables in rituximab-treated refractory myositis patients. Methods: We analyzed data for 200 myositis patients (76 with adult polymyositis (PM), 76 with adult dermatomyositis (DM), and 48 with juvenile (DM)) in the rituximab in myositis trial. Clinical improvement is defined as the change from baseline to 24 weeks in Physician Global Visual Analog Scale (VAS). We analyze the association of baseline variables with improvements: demographics, myositis subtype, clinical and laboratory parameters, autoantibody status, and interferon (IFN)- regulated chemokines. Multivariable linear regression models are developed by using stepwise variable selection methods. Results: A “base” multivariable model to predict improvement with clinical and laboratory variablesonly is built with modest predictive ability (adjusted R2 = 0.21). This model includes two significant factors at baseline: Physician Global VAS and Muscle Disease Activity VAS. A “final” multivariable model to predict improvement including non-standard laboratory measures is developed and demonstrated better predictive ability (adjusted R2 = 0.32). This model includes Physician Global VAS, IFN chemokine score and IL-2 levels. The “final” model explained 11% more variability than the “base” model. Conclusions: Changes in disease activity over time following treatment with rituximab in refractory myositis can be predicted. These models can be clinically useful to optimize treatment selection in myositis.展开更多
文摘Background Juvenile dermatomyositis(JDM)is a chronic autoimmune disease characteristic by inflammation of small vessels within the skin,muscle and vital organs.But the clinical features and treatment of JDM have not been fully clarified.Data sources Databases underwent through PubMed for articles about the clinical features,myositis-specific antibodiesof JDM and its treatment,and we selected publications written in English which were relevant to the topic of this review.Results Clinical features and myositis-specific antibodies may predict the severity and prognosis of disease.Although the mortality rate has been lower with traditional treatments,such as corticosteroid,intravenous immunoglobulin,and diseasemodifying anti-rheumatic drugs such as methotrexate,their usages are variable.Novel biological therapies seem to be effective for refractory JDM patients,but more clinical trials are necessary.Conclusions JDM is a sever disease of childhood.We need to better understand recent advances of JDM in the context of clinical features including skin manifestations,muscle weakness and organ damage,myositis-specific antibodies and their associated outcomes and the treatment of disease.
文摘This study investigated the role of mast cells in canine cutaneous vascular tumors, and is the first such study to distinguish between tumors arising in the dermis versus the subcutis. Mast cell numbers in canine cutaneous hemangiomas (HA) and hemangiosarcomas (HSA) were evaluated to identify a relationship between mast cells, tumor type (HA, HSA), histologic location (dermis, subcutis) and tumor recurrence. One hundred and sixty-seven biopsies from 148 dogs were evaluated. Using only one biopsy from each dog, mast cell counts (MCC) for each tumor (n = 148) were obtained by averaging the number of mast cells counted in ten 400× fields. A significant difference in mean MCC was found only between tumor types, with HA having more mast cells than HSA (4.2 ± 4.2 vs. 2.2 ± 2.6;p < 0.001). No significant difference in mean MCC existed between tumors that recurred and those that did not. There was no difference in recurrence rate between tumor type or histologic location. Our results indicate that benign HA contain more mast cells than malignant endothelial cell tumors, regardless of histologic location;whether this is a cause or effect relationship remains to be determined.
文摘Background: Response to rituximab so far is unpredictable in patients with refractory myositis. Predictive models of clinical improvement are developed using clinical, laboratory, and gene expression/cytokine/chemokine variables in rituximab-treated refractory myositis patients. Methods: We analyzed data for 200 myositis patients (76 with adult polymyositis (PM), 76 with adult dermatomyositis (DM), and 48 with juvenile (DM)) in the rituximab in myositis trial. Clinical improvement is defined as the change from baseline to 24 weeks in Physician Global Visual Analog Scale (VAS). We analyze the association of baseline variables with improvements: demographics, myositis subtype, clinical and laboratory parameters, autoantibody status, and interferon (IFN)- regulated chemokines. Multivariable linear regression models are developed by using stepwise variable selection methods. Results: A “base” multivariable model to predict improvement with clinical and laboratory variablesonly is built with modest predictive ability (adjusted R2 = 0.21). This model includes two significant factors at baseline: Physician Global VAS and Muscle Disease Activity VAS. A “final” multivariable model to predict improvement including non-standard laboratory measures is developed and demonstrated better predictive ability (adjusted R2 = 0.32). This model includes Physician Global VAS, IFN chemokine score and IL-2 levels. The “final” model explained 11% more variability than the “base” model. Conclusions: Changes in disease activity over time following treatment with rituximab in refractory myositis can be predicted. These models can be clinically useful to optimize treatment selection in myositis.
