Clinical characteristics and management of clinically amyopathic juvenile dermatomyositis across four academic centers

2021 ◽  
Author(s):  
Flora Bradley ◽  
Michelle L. Bayer ◽  
Dominic O. Co ◽  
Yvonne Chiu ◽  
Adam M. Huber ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Juvenile Dermatomyositis

scholarly journals Clinical characteristics of children with juvenile dermatomyositis recruited within the first 7 months of the CARRAnet registry

2012 ◽  
Vol 10 (S1) ◽  
Author(s):  
Mark F Hoeltzel ◽  
◽  
Mara L Becker ◽  
Angela B Robinson ◽  
Brian M Feldman ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Juvenile Dermatomyositis

scholarly journals Clinical characteristics and poor predictors of anti-NXP2 antibody-associated Chinese JDM children

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Xinning Wang ◽  
Yuchuan Ding ◽  
Zhixuan Zhou ◽  
Jun Hou ◽  
Yingjie Xu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Muscle Weakness ◽  
Poor Prognosis ◽  
Clinical Characteristics ◽  
Stem Cell Transplant ◽  
Juvenile Dermatomyositis ◽  
Autologous Stem Cell Transplant ◽  
Skin Ulcer ◽  
Cell Transplant ◽  
Gastrointestinal Involvement

Abstract Background Juvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The anti-NXP2 antibody is one of the most common antibodies and muscle ischaemia associated with NXP2 autoantibodies was a severe subtype of JDM. Further information is needed regarding clinical characteristics and factors associated with poor prognosis. But there are no reports about clinical characteristics and high risk factor of poor prognosis. For the first time, we introduced the clinical characteristics and poor predictors of anti-NXP2 antibody-associated juvenile dermatomyositis in Chinese children. Methods Twenty-six patients with anti-NXP2 antibody-related JDM from 85 JDM Chinese patients were diagnosed from January 2016 to November 2019. Logistic regression was used to analyze the risk factors for refractory cases and mortality. Results The ratio of male to female was 1:1.9. The median age of onset was 4.5 (1–13) years. Twenty-four cases (92.3%) had rash and muscle weakness. Treatments included glucocorticoids, immunosuppressive agents, biological agents (7 cases), plasma exchange, Janus kinase inhibitor (7 cases) and autologous stem cell transplant (1 case). Refractory JDM patients (11/26, 42.3%) were associated with edema, skin ulcer, muscle strength<=grade 3, CD4/CD8 ratio < 1.4 and ferritin > 200μg/ml. Among 6 cases (6/26, 23.1%) with severe gastrointestinal involvement, 5 cases died and 1 case survived after autologous stem cell transplant (ASCT). The risk factors for gastrointestinal involvement and mortality were edema, skin ulcer, severe muscle weakness (dysphagia/ hoarseness/ soft voice), BMI < 15 and ANA positive. Conclusions Edema, skin ulcer and severe muscle weakness predicted refractory disease, GI involvement, and mortality in anti-NXP2 antibody-positive JDM of Chinese children. Decreased CD4/CD8 ratio and high ferritin related with refractory cases, and very low BMI and ANA (+) are probably, associated with gastrointestinal involvement and mortality. Trial registration http://www.chictr.org.cn/showproj.aspx?proj=49846.

scholarly journals Clinical Characteristics and Treatment Response of Juvenile Dermatomyositis

2016 ◽  
Vol 24 (4) ◽  
pp. 232-239
Author(s):  
우혜원 ◽  
김수연 ◽  
최선아 ◽  
채종희 ◽  
임병찬 ◽  
...  
Keyword(s):  
Treatment Response ◽  
Clinical Characteristics ◽  
Juvenile Dermatomyositis

scholarly journals Anti-Ro52 autoantibodies are associated with interstitial lung disease and more severe disease in patients with juvenile myositis

2019 ◽  
Vol 78 (7) ◽  
pp. 988-995 ◽  
Author(s):  
Sara Sabbagh ◽  
Iago Pinal-Fernandez ◽  
Takayuki Kishi ◽  
Ira N Targoff ◽  
Frederick W Miller ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Clinical Characteristics ◽  
Juvenile Dermatomyositis ◽  
Severe Disease ◽  
Trna Synthetase ◽  
Disease Course

ObjectivesAnti-Ro52 autoantibodies are associated with more severe interstitial lung disease (ILD) in adult myositis patients with antiaminoacyl transfer (t)RNA synthetase autoantibodies. However, few studies have examined anti-Ro52 autoantibodies in juvenile myositis. The purpose of this study was to define the prevalence and clinical features associated with anti-Ro52 autoantibodies in a large cohort of patients with juvenile myositis.MethodsWe screened sera from 302 patients with juvenile dermatomyositis (JDM), 25 patients with juvenile polymyositis (JPM) and 44 patients with juvenile connective tissue disease–myositis overlap (JCTM) for anti-Ro52 autoantibodies by ELISA. Clinical characteristics were compared between myositis patients with and without anti-Ro52 autoantibodies.ResultsAnti-Ro52 autoantibodies were found in 14% patients with JDM, 12% with JPM and 18% with JCTM. Anti-Ro52 autoantibodies were more frequent in patients with antiaminoacyl tRNA synthetase (64%, p<0.001) and anti-MDA5 (31%, p<0.05) autoantibodies. After controlling for the presence of myositis-specific autoantibodies, anti-Ro52 autoantibodies were associated with the presence of ILD (36% vs 4%, p<0.001). Disease course was more frequently chronic, remission was less common, and an increased number of medications was received in anti-Ro52 positive patients.ConclusionsAnti-Ro52 autoantibodies are present in 14% of patients with juvenile myositis and are strongly associated with anti-MDA5 and antiaminoacyl tRNA synthetase autoantibodies. In all patients with juvenile myositis, those with anti-Ro52 autoantibodies were more likely to have ILD. Furthermore, patients with anti-Ro52 autoantibodies have more severe disease and a poorer prognosis.

scholarly journals Clinical Characteristics of Children With Juvenile Dermatomyositis: The Childhood Arthritis and Rheumatology Research Alliance Registry

2014 ◽  
Vol 66 (3) ◽  
pp. 404-410 ◽  
Author(s):  
Angela Byun Robinson ◽  
Mark F. Hoeltzel ◽  
Dawn M. Wahezi ◽  
Mara L. Becker ◽  
Elizabeth A. Kessler ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Juvenile Dermatomyositis ◽  
Rheumatology Research ◽  
Childhood Arthritis ◽  
Research Alliance

Juvenile dermatomyositis: clinical characteristics and the relatively high risk of interstitial lung disease

2007 ◽  
Vol 17 (5) ◽  
pp. 413-417 ◽  
Author(s):  
Yoichi Morinishi ◽  
Tsutomu Oh-Ishi ◽  
Tomoyuki Kabuki ◽  
Kosuke Joh
Keyword(s):  
High Risk ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Characteristics ◽  
Juvenile Dermatomyositis

scholarly journals Clinical Characteristics and Poor Predictors of Anti-NXP2 Antibody-Associated Chinese JDM Children

2020 ◽  
Author(s):  
Xinning Wang ◽  
Yuchuan ding ◽  
Zhixuan Zhou ◽  
Jun Hou ◽  
Yingjie Xu ◽  
...  
Keyword(s):  
Risk Factors ◽  
High Risk ◽  
Muscle Weakness ◽  
Poor Prognosis ◽  
Clinical Characteristics ◽  
Juvenile Dermatomyositis ◽  
Skin Ulcer ◽  
Janus Kinase ◽  
Chinese Children ◽  
Gastrointestinal Involvement

Abstract Background. Juvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The subtype of anti-NXP2 antibody-associated JDM is the leading cause of death in JDM, but there are no reports about clinical characteristics and high risk factors of poor prognosis. For the first time, we introduced the clinical characteristics and poor predictors of anti-NXP2 antibody-associated juvenile dermatomyositis in Chinese children.Methods. Twenty-six patients with anti-NXP2 antibody-related JDM from 85 JDM patients diagnosed from January 2016 to November 2019 were involved. Logistic regression was used to analyze the risk factors for refractory cases and death.Results. The ratio of male to female was 9:17. The median age of onset was 4.5 (1–13) years. Twenty-four cases (92.3%) had rash and muscle weakness. Treatments included glucocorticoids, immunosuppressive agents, biological agents (7 cases), plasma exchange, Janus kinase inhibitor (7 cases) and autologous stem cell transplant (1 case). Eleven cases (11/26, 42.3%) were refractory JDM associated with edema, skin ulcer, muscle strength < = grade 3, CD4/CD8 ratio < 1.4 and SF > 200ug/ml. Among 6 cases (6/26, 23.1%) with severe gastrointestinal involvement, 5 cases died and 1 case survived after ASCT. The risk factors for gastrointestinal involvement and death were edema, skin ulcer, severe muscle weakness (Dysphagia/Hoarseness/Lower voice), BMI < 15 and ANA positive.Conclusions. Anti-NXP2 antibody-positive JDM of Chinese children was characterized by rash and severe muscle weakness. Edema, skin ulcer and severe muscle weakness predicted refractory and poor prognosis. Decreased CD4/CD8 ratio and high SF related with refractory cases, and very low BMI and ANA (+) predicted high risk of gastrointestinal involvement and death.

scholarly journals The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)--clinical characteristics of children recruited within the first 5 yr

Rheumatology ◽  
2006 ◽  
Vol 45 (10) ◽  
pp. 1255-1260 ◽  
Author(s):  
L. J. McCann ◽  
A. D. Juggins ◽  
S. M. Maillard ◽  
L. R. Wedderburn ◽  
J. E. Davidson ◽  
...  
Keyword(s):  
Clinical Characteristics ◽  
Juvenile Dermatomyositis ◽  
National Registry

scholarly journals Clinical characteristics and poor predictors of anti-NXP2 antibody-associated Chinese JDM children

2020 ◽  
Author(s):  
Xinning Wang ◽  
Yuchuan ding ◽  
Zhixuan Zhou ◽  
Jun Hou ◽  
Yingjie Xu ◽  
...  
Keyword(s):  
Risk Factors ◽  
Muscle Weakness ◽  
Poor Prognosis ◽  
Clinical Characteristics ◽  
Stem Cell Transplant ◽  
Juvenile Dermatomyositis ◽  
Autologous Stem Cell Transplant ◽  
Skin Ulcer ◽  
Cell Transplant ◽  
Gastrointestinal Involvement

Abstract Background. Juvenile dermatomyositis (JDM) is a rare and sometimes fatal disease in children. The anti-NXP2 antibody is one of the most common antibodies and muscle ischaemia associated with NXP2 autoantibodies was a severe subtype of JDM.Further information is needed regarding clinical characteristics and factors associated with poor prognosis. But there are no reports about clinical characteristics and high risk factor of poor prognosis. For the first time, we introduced the clinical characteristics and poor predictors of anti-NXP2 antibody-associated juvenile dermatomyositis in Chinese children.Methods. Twenty-six patients with anti-NXP2 antibody-related JDM from 85 JDM Chinese patients were diagnosed from January 2016 to November 2019 . Logistic regression was used to analyze the risk factors for refractory cases and mortality.Results. The ratio of male to female was 1:1.9.. The median age of onset was 4.5 (1-13) years. Twenty-four cases (92.3%) had rash and muscle weakness. Treatments included glucocorticoids, immunosuppressive agents, biological agents (7 cases), plasma exchange, Janus kinase inhibitor (7 cases) and autologous stem cell transplant (1 case). Refractory JDM patients (11/26,42.3%) were associated with edema, skin ulcer, muscle strength<=grade 3, CD4/CD8 ratio <1.4 and ferritin >200ug/ml. Among 6 cases (6/26, 23.1%) with severe gastrointestinal involvement, 5 cases died and 1 case survived after autologous stem cell transplant (ASCT). The risk factors for gastrointestinal involvement and mortality were edema, skin ulcer, severe muscle weakness (dysphagia/ hoarseness/ soft voice), BMI<15 and ANA positive.Conclusions. . Edema, skin ulcer and severe muscle weakness predicted refractory disease, GI involvement, and mortality in anti-NXP2 antibody-positive JDM of Chinese children. Decreased CD4/CD8 ratio and high ferritin related with refractory cases, and very low BMI and ANA (+) are probably, associated with gastrointestinal involvement and mortality .

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