Clinical Characteristics of Juvenile Idiopathic Inflammatory Myopathy and Comparison With Adult Patients

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Jesús Loarce-Martos ◽  
Carmen Larena ◽  
M. Ángeles Blázquez ◽  
Beatriz E. Joven ◽  
Patricia E. Carreira ◽  
...  
2009 ◽  
Vol 36 (11) ◽  
pp. 2547-2551 ◽  
Author(s):  
GRACIELA ESPADA ◽  
JOSE A. MALDONADO COCCO ◽  
NOREEN FERTIG ◽  
CHESTER V. ODDIS

Objective.Autoantibodies are frequently found in adult patients with polymyositis (PM), dermato-myositis (DM), and overlap myositis disorders. They are less common in pediatric patients with myositis. We investigated the autoantibody pattern in a pediatric Argentine Caucasian cohort to characterize novel autoantibodies.Methods.Sera from children that satisfied published criteria for idiopathic inflammatory myopathy were analyzed for autoantibodies by RNA and protein immunoprecipitation and immunoblotting techniques. Routine myositis-specific and myositis-associated autoantibodies as well as autoantibody specificities were determined.Results.We tested sera from 64 consecutive pediatric myositis patients, including 40 with juvenile DM, 7 with juvenile PM, and 17 with overlap myositis syndromes. Sixteen (25%) patients were found to have anti-MJ autoantibody exclusively, which appears to identify a subset of pediatric myositis patients with severe disease characterized by muscle contractures and atrophy and significant compromise of functional status. Fourteen (22%) patients were found to have an antibody targeting 2 proteins of 155 and 140 kDa. Other myositis-specific autoantibodies were uncommon in this pediatric cohort.Conclusion.A newly recognized autoantibody, anti-MJ, was the most common antibody found in this Argentine pediatric cohort. The clinical features indicated that this antibody is distinct from other reported antibodies in pediatric patients with myositis.


2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
E Bollache ◽  
AT Huber ◽  
J Lamy ◽  
E Afari ◽  
TM Bacoyannis ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background. Recent studies revealed the ability of MRI T1 mapping to characterize myocardial involvement in both idiopathic inflammatory myopathy (IIM) and acute viral myocarditis (AVM), as compared to healthy controls. However, neither myocardial T1 nor T2 maps were able to discriminate between IIM and AVM patients, when considering conventional myocardial mean values and derived indices such as lambda and extracellular volume. Purpose. To investigate the ability of T1 mapping-derived texture analysis to differentiate IIM from AVM. Methods. Forty patients, 20 with IIM (51 ± 17 years, 9 men) and 20 with AVM (34 ± 13 years, 16 men) underwent 1.5T MRI T1 mapping using a modified Look-Locker inversion-recovery sequence before and 15 minutes after injection of a gadolinium contrast agent. After manual delineation of endocardial and epicardial borders and co-registration of all inversion time images, native and post-contrast T1 maps were estimated. Myocardial texture analysis was performed on native T1 maps. Textural features such as: autocorrelation, contrast, dissimilarity, energy and sum entropy were used to build a least squares-based linear regression model. Finally, receiver operating characteristic (ROC) analysis was used to investigate the ability of such texture features score to classify IIM vs. AVM patients, compared to the performance of mean myocardial T1. A Wilcoxon rank-sum test was also used to test difference significance between groups. Results. Both native and post-contrast mean myocardial T1 values were comparable between IIM (native: 1022 ± 43 ms; post-contrast: 319 ± 44 ms) and AVM (1056 ± 59 ms, p = 0.07; 318 ± 35 ms, p = 0.90, respectively) groups. Results of ROC analyses are provided in the Table, indicating that a better discrimination between IIM and AVM patients was obtained when using texture features, with higher AUC and accuracy than mean T1 values (Figure). Conclusion. Texture analysis derived from MRI T1 maps without contrast agent injection was able to discriminate between IIM and AVM with higher accuracy, sensitivity and specificity than conventional T1 indices. Such analysis could provide a useful myocardial signature to help diagnose and manage cardiac alterations associated with IIM in patients presenting with myocarditis and primarily suspected of AVM. Table Area under curve (AUC) Accuracy Sensitivity Specificity Native T1 0.67 0.70 0.65 0.75 Post-contrast T1 0.49 0.60 0.25 0.95 Texture features score 0.85 0.82 0.90 0.75 ROC analyses for classification between IIM and AVM patients Abstract Figure


Author(s):  
Tatiana Cobo-Ibáñez ◽  
Carlos Sánchez-Piedra ◽  
Laura Nuño-Nuño ◽  
Iván Castellví ◽  
Irene Carrión-Barberà ◽  
...  

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