Faculty Opinions recommendation of Histological heterogeneity in a large clinical cohort of juvenile idiopathic inflammatory myopathy: analysis by myositis autoantibody and pathological features.

Author(s):  
Manabu Fujimoto ◽  
Naoko Okiyama
2020 ◽  
Author(s):  
Lu Zhang ◽  
Hanbo Yang ◽  
Jieping Lei ◽  
Qinglin Peng ◽  
Hongxia Yang ◽  
...  

Abstract BackgroudAnti-mitochondrial antibodies (AMAs) can be detected in some idiopathic inflammatory myopathy (IIM) patients. We aimed to investigate the clinical features of IIM patients with AMAs.Methods We retrospectively analysed consecutive 1,167 patients with IIM for AMAs-associated myositis and compared them to age- and sex-matched AMA-negative patients. ResultsTwenty-nine patients (2.5%) were identified with AMAs-positive myositis; eight of them had primary biliary cholangitis (PBC). There were no significant differences in skin rash, dysphagia, interstitial lung disease, and muscle strength between AMAs-positive patients and disease controls. 12/23(52.2%) cases showed immune-mediated necrotizing myopathy (IMNM)-like pathological features. Among AMAs-positive patients, 11 of 16 patients with isolated anti-AMA were classified as IMNM which was significantly higher than that of patients with coexistent anti-AMAs and myositis-specific antibodies (p=0.026). Moreover, AMAs-positive patients had a significantly higher cardiac involvement ratio (P<0.001) compared to controls. Comparsion in AMAs-positive IIM patients show the incidence of abnormal echocardiography findings was significantly higher in patients without primary biliary cholangitis (PBC) than in patients with PBC(P=0.009). Patients without heart abnormalities took significantly less time to achieve disease remission and prednisone tapering to <10 mg than patients with heart abnormalities (P<0.001 and P=0.001, respectively).ConclusionsIMNM was a major histopathological finding in IIM patients with isolated anti-AMAs antibody. AMAs was significantly associated with cardiac involvement in IIM. PBC seemed to be a protective factor for abnormal echocardiography findings in AMAs-positive patients. Patients without heart involvement took less time to achieve disease remission and prednisone tapering off.


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 ◽  
...  

2002 ◽  
Vol 21 (5) ◽  
pp. 391-396 ◽  
Author(s):  
C.-S. Lee ◽  
T.-L. Chen ◽  
C.-Y. Tzen ◽  
F.-J. Lin ◽  
M.-J. Peng ◽  
...  

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