BACKGROUND Juvenile dermatomyositis(JDM)is an idiopathic inflammatory myopathy that occurs in childhood.It is characterized by muscle weakness and a characteristic rash.Previous literature reports have rarely describe...BACKGROUND Juvenile dermatomyositis(JDM)is an idiopathic inflammatory myopathy that occurs in childhood.It is characterized by muscle weakness and a characteristic rash.Previous literature reports have rarely described JDM with severe skin ulcers and infections.CASE SUMMARY Herein,we describe a case of a 2-year-old female patient who suffered from JDM,whose myositis-specific autoantibodies were positive for anti-nuclear matrix protein 2 antibody,with progressively worsening skin ulcers and severe infections.The patient was treated with glucocorticoids and various immunosuppressants.Nevertheless,further progression of the disease and the combination of primary disease and severe infection in the later period were fatal.CONCLUSION In children,anti-nuclear matrix protein 2+JDM combined with skin ulcers often indicates severe disease.In such cases,personalized treatment for the primary disease and infection prevention and control are essential.展开更多
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
Background The underlying etiology of juvenile dermatomyositis(JDM)is unknown.T cell deficiency as well as Epstein-Barr virus(EBV)infection had been suspected to be involved in the pathogenesis,but it has been poorly ...Background The underlying etiology of juvenile dermatomyositis(JDM)is unknown.T cell deficiency as well as Epstein-Barr virus(EBV)infection had been suspected to be involved in the pathogenesis,but it has been poorly evaluated in JDM patients.Methods This study described the traits of T and B lymphocyte subsets in newly onset JDM patients and the incidence of EBV infection in JDM patients compared with match controls.Newly developed JDM patients from 2014 to 2018 were included in the study.Lymphocytes with different markers(CD3+,CD3+CD4+,CD3+CD8+,CD3-CD19+and CD3-CD16+CD56+)were tested with flow cytometry in the first admission or after 6 months of treatment.Statistical analysis was conducted to compare the EBV infection in the group of JDM patients and controls.Results We observed that JDM patients had higher positive rate of Epstein-Barr nuclear antigen-immunoglobulin G(IgG)(P<0.0001)as well as EBV capsid antigen-IgG(P<0.05)than normal controls.CD3-CD16+CD56+lymphocyte was found to be extremely low in early stage of JDM patients,but increased after 6 months of treatment(P=0.0091).Conclusions The level of CD3-CD16+CD56+cells may associate with the clinical course of JDM.EBV may act as an environmental factor predisposing patients to the development of JDM.展开更多
文摘BACKGROUND Juvenile dermatomyositis(JDM)is an idiopathic inflammatory myopathy that occurs in childhood.It is characterized by muscle weakness and a characteristic rash.Previous literature reports have rarely described JDM with severe skin ulcers and infections.CASE SUMMARY Herein,we describe a case of a 2-year-old female patient who suffered from JDM,whose myositis-specific autoantibodies were positive for anti-nuclear matrix protein 2 antibody,with progressively worsening skin ulcers and severe infections.The patient was treated with glucocorticoids and various immunosuppressants.Nevertheless,further progression of the disease and the combination of primary disease and severe infection in the later period were fatal.CONCLUSION In children,anti-nuclear matrix protein 2+JDM combined with skin ulcers often indicates severe disease.In such cases,personalized treatment for the primary disease and infection prevention and control are essential.
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.
基金This work was supported by Zhejiang Medical and Health Science and Technology Project(No.2017KY440).
文摘Background The underlying etiology of juvenile dermatomyositis(JDM)is unknown.T cell deficiency as well as Epstein-Barr virus(EBV)infection had been suspected to be involved in the pathogenesis,but it has been poorly evaluated in JDM patients.Methods This study described the traits of T and B lymphocyte subsets in newly onset JDM patients and the incidence of EBV infection in JDM patients compared with match controls.Newly developed JDM patients from 2014 to 2018 were included in the study.Lymphocytes with different markers(CD3+,CD3+CD4+,CD3+CD8+,CD3-CD19+and CD3-CD16+CD56+)were tested with flow cytometry in the first admission or after 6 months of treatment.Statistical analysis was conducted to compare the EBV infection in the group of JDM patients and controls.Results We observed that JDM patients had higher positive rate of Epstein-Barr nuclear antigen-immunoglobulin G(IgG)(P<0.0001)as well as EBV capsid antigen-IgG(P<0.05)than normal controls.CD3-CD16+CD56+lymphocyte was found to be extremely low in early stage of JDM patients,but increased after 6 months of treatment(P=0.0091).Conclusions The level of CD3-CD16+CD56+cells may associate with the clinical course of JDM.EBV may act as an environmental factor predisposing patients to the development of JDM.
