Polymyositis, Dermatomyositis, and Inclusion Body Myositis

Holton, Janice L.; Wedderburn, Lucy R.; Hanna, Michael G.

doi:10.1002/9781118635469.ch33

Cited by 4 publications

(4 citation statements)

References 67 publications

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“…Studies showed that more than 2 thirds of patients developed a chronic course and 4.1% of patients died [ 7 , 8 ], patients have to taken drugs for years and some depends on wheelchair, which seriously affects patients’ quality of life and social participation. MSA is potentially useful biomarker for it is associated with different clinical phenotypes [ 9 , 10 ]. In children with JDM, anti-MDA5, anti-TIF-1γ and anti-NXP2 are the most common MSAs [ 11 – 15 ].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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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.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2021

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“…MSA is potentially useful biomarker for it is associated with different clinical phenotypes. 7,8 In children with JDM, anti-MDA5, anti-TIF-1γ and anti-NXP2 are the most common MSAs. [9][10][11][12][13] The reports from UK and Argentina showed anti-NXP2 antibody were detected in 23% and 25% respectively of patients with JDM.…”

Section: Discussionmentioning

confidence: 99%

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

Wang

Ding

Zhou

et al. 2020

Preprint

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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.

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“…[7][8][9] A few reports of persistent CNFs challenge the notion that these are markers of ongoing repair, suggesting that central nucleation could be a permanent pathological feature signifying past regeneration events. [10][11][12][13] However, they could also be explained by a lengthy nuclear repositioning mechanism that lags behind the recovery of fiber size and function by several months.…”

Section: Discussionmentioning

confidence: 99%

“…Cases were identified from the South Australian Myositis Database (SAMD), a statewide Microsoft Access registry (ethics approved by Ethics Committee of Royal Adelaide Hospital, Protocol 151012) containing all South Australian patients with biopsy‐confirmed IM subsequent to 1980. All adult muscle biopsies in South Australia are assessed in a single laboratory at SA Pathology using accepted histopathologic criteria for diagnosis and regular peer review of all biopsies. Data recorded in the database includes demographic, histologic, and clinical details, such as medications used at time of muscle biopsy and comorbidities.…”

Section: Methodsmentioning

confidence: 99%

Proton pump inhibitors are not associated with inflammatory myopathies: A case control study

et al. 2018

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Polymyositis, Dermatomyositis, and Inclusion Body Myositis

Cited by 4 publications

References 67 publications

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

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

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

Proton pump inhibitors are not associated with inflammatory myopathies: A case control study

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