Assessment of serum sST2 for cardiac involvement in idiopathic inflammatory myopathies.

Idiopathic inflammatory myopathies (IIM) is a group of heterogeneous autoimmune systemic diseases, which not only involve skeletal muscle but also myocardium. Cardiac involvement in IIM, which eventually develops into heart failure, is difficult to identify by conventional examinations at early stage. The aim of this study was to investigate if multi-parametric cardiac magnetic resonance (CMR) imaging can screen for early cardiac involvement in IIM, compared with clinical score (Myositis Disease Activity Assessment Tool, MDAAT). Forty-nine patients of IIM, and 25 healthy control subjects with comparable age-range and sex-ratio were enrolled in this study. All subjects underwent CMR examination, and multi-slice short-axis and 4-chamber cine MRI were acquired to evaluate biventricular global circumferential strain (GCS) and global longitudinal strain (GLS). Native T1 and T2 mapping were performed, and post-contrast T1 mapping and LGE were acquired after administration of contrast. A CMR score was developed from native T1 mean and T2 mean for the identification of cardiac involvement in the IIM cohort. Using contingency tables MDAAT and CMR were compared and statistically analyzed using McNemar test. McNemar’s test revealed no significant difference between CMR score and MDAAT (p = 0.454). CMR score had potential to screen for early cardiac involvement in IIM patients, compared to MDAAT.

AB0652 QTC INTERVAL PROLONGATION IN A SCANDINAVIAN COHORT OF PATIENTS WITH IDIOPATHIC INFLAMMATORY MYOPATHIES AND SYSTEMIC SCLEROSIS: CORRELATIONS WITH CLINICAL VARIABLES

Cardiac involvement is common in idiopathic inflammatory myopathy (IIM) but often subclinical. Cardiac magnetic resonance (CMR) is a promising tool in detecting cardiac involvement in patients with IIM. The aim of this study was to assess cardiac involvement in IIM patients by CMR feature tracking (CMR-FT). Thirty-seven IIM patients and 25 controls were enrolled in this retrospective study. The left ventricular (LV) functional parameters such as volume and ejection fraction were measured. Global and regional LV peak strain (PS) in radial, circumferential and longitudinal directions were derived from cine images. Left atrial (LA) volume, longitudinal strain and strain rate (SR) parameters and LA reservoir function, conduit function and booster pump function were assessed, respectively. IIM patients with preserved LVEF showed significantly reduced global and regional LV PS in longitudinal direction (all p < 0.05). Compared with controls, LA reservoir and conduit function were significantly impaired in IIM patients (all p < 0.05). The global LV longitudinal PS, LAVpre-ac and SRe were independent predictors of IIM. By Pearson's correlation analysis, the LV global radial, circumferential and longitudinal PS were all correlated to LVEF in IIM patients (r = 0.526, p < 0.001 vs. r = -0.514, p < 0.001 vs. r = - 0.288, p = 0.023). CMR-FT based LV and LA deformation performance could early detect cardiac involvement in IIM patients with preserved LVEF.

Objective We aimed to investigate the cardiovascular profile, including risk factors and cardiovascular abnormalities, in patients with idiopathic inflammatory myopathies (IIMs). Method In this cross-sectional study, 109 IIM patients and 20 age- and gender-matched healthy controls were enrolled and underwent electrocardiographic and transthoracic echocardiographic examinations. We analysed blood levels of cardiac troponin I (cTnI) and N-terminal pro-brain natriuretic peptide (NT-proBNP), assessed IIM disease-specific features, and evaluated the medical history of cardiovascular risk factors. IIM patients were stratified into two groups: those with previous cardiac involvement and those without. Results IIM patients had a higher body mass index (BMI) and a greater prevalence of diabetes mellitus and dyslipidaemia than healthy controls (p = 0.023, p = 0.024, and p = 0.042, respectively). They also showed significantly higher rates of arrhythmia, cardiac axis deviation, negative T-waves, and suspected pulmonary hypertension, along with elevated NT-proBNP levels (p = 0.041, p = 0.004, p = 0.041, p = 0.012, and p = 0.034, respectively). A significantly higher proportion (p = 0.037) of immune-mediated necrotizing myopathy (IMNM) subtype (50%) was found among IIM with previous cardiac involvement compared to those without (20%). cTnI levels were significantly higher in IIM with cardiac involvement than in IIM without cardiac involvement (p = 0.009). Conclusions Cardiovascular complications in patients with IIM may result from an increased prevalence of traditional cardiovascular risk factors, such as higher BMI, diabetes mellitus, and dyslipidaemia, and/or from direct cardiac involvement, such as previous myocarditis. Cardiac involvement in IIM is notably associated with the IMNM subtype.

ObjectiveAnti-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. MethodsWe retrospectively analysed 1,167 consecutive patients with IIM for AMA-associated myositis and compared them to age- and gender-matched AMA-negative IIM patients. ResultsTwenty-nine patients (2.5%) were identified with AMA-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 AMA-positive patients and AMA-negative patients. Of 23 cases, 12 (52.2%) showed immune-mediated necrotizing myopathy (IMNM)-like pathological features. amongst AMA-positive patients, 11 of 16 patients with isolated anti-AMAs were classified as IMNM which was significantly higher than that of patients with coexistent anti-AMAs and myositis-specific antibodies (p = 0.026). Moreover, subclinical cardiac involvement was significantly more common in AMA-positive patients than in AMA-negative patients (21/29 VS 33/116, p<0.001). In addition, patients without PBC had a significantly higher incidence of abnormal echocardiography findings than that of 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 = 0.000 and p = 0.001, respectively). ConclusionsIMNM was a major histopathological finding in IIM patients with isolated AMAs. AMAs were significantly associated with subclinical cardiac involvement in IIM. PBC seemed to be a protective factor for abnormal echocardiography findings in AMA-positive patients. Patients without heart involvement took less time to achieve disease remission and prednisone tapering off.

Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune diseases that includes the main subtypes dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, and inclusion body myositis. IIMs are characterized by the involvement of skeletal muscle and multiple organs, including the heart. This review summarizes the pathology, prevalence, biomarkers, imaging and treatment of cardiac involvement in patients with IIMs. The cardiac involvement in these patients is usually subclinical and rarely considered as the main clinical feature at the time of initial consultation, with a prevalence ranging from 4% to 26%. However, it results in a worse prognosis and represents the main cause of mortality in patients with IIMs. The selection of specific serum cardiac biomarkers is essential for the early detection of cardiac involvement in patients with IIMs, such as cardiac troponin I (cTnI), which is preferred over cardiac troponin T (cTnT), followed by diagnostic evaluations including electrocardiography (ECG), echocardiography (ECHO), and cardiac magnetic resonance imaging (CMR). The combination of glucocorticoids, immunosuppressants, and conventional cardiac medications is effective for the management of IIM patients with confirmed cardiac involvement.

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

The objectives of this study are to analyze the association between anti-mitochondrial antibody (AMA) and cardiac involvement in idiopathic inflammatory myopathy (IIM) and to evaluate the diagnostic value of AMA for cardiac involvement in IIM patients. We conducted acomprehensive search in PubMed, Web of Science, EMBASE, and the Cochrane Library to identify English-language studies published before November19, 2021. Stata12.0 software (Stata Corp., College Station, TX, USA) was used for the statistical analyses. We used the sensitivity, specificity, positive likelihood ratio (PLR), negative likelihood ratio (NLR), and summary receiver operating characteristic (SROC) curve to evaluate the diagnostic value of AMA for cardiac involvement in IIM patients. Statistical heterogeneity of studies was assessed using the I2 statistic with 95% confidence intervals (95% CIs). Seven studies were included in the final analyses, with atotal of 2308IIM patients (including 171 AMA-positive and 2137 AMA-negative patients). The pooled sensitivity of AMA for cardiac involvement in IIM patients was 0.29 (95% CI: 0.19-0.43) and specificity was 0.92 (95% CI: 0.88-0.96). The pooled PLR was 3.9 (95% CI: 2.82-5.38), NLR was 0.76 (95% CI: 0.66-0.88), and the diagnostic odds ratio (DOR) was 5 (95% CI: 3-7). The area under the SROC curve was 0.76 (95% CI: 0.72-0.79). The overall diagnostic value of AMA may not be very high for cardiac involvement in IIM patients.

To investigate whether cardiovascular magnetic resonance (CMR) T1 mapping and strain parameters can detect early histological and functional myocardial changes in idiopathic inflammatory myopathy (IIM) with negative late gadolinium enhancement (LGE) and preserved ejection fraction. Thirty consecutive patients with IIM (41.5 ± 15.4 years, 24 females) who did not have LGE or reduced left ventricular ejection fraction (LVEF) and 30 age- and gender-matched healthy controls (40.6 ± 14.2 years, 20 females) were recruited. Patients with IIM were further classified into two subgroups according to high-sensitivity cardiac troponin I (hs-cTnI) values: elevated hs-cTnI subgroup (n = 10) and normal hs-cTnI subgroup (n = 20). Myocardial native T1 values, extracellular volume (ECV) fractions, and strain parameters were analyzed in patients with IIM and healthy controls. Compared with healthy controls, patients with IIM had significantly prolonged native T1 values and increased ECV in each LV segment (p < 0.05). In further subgroup analysis, LV mid-slice native T1 values had the most power to discriminate between patients with elevated hs-cTnI and healthy controls (area under the curve = 0.92). There was no significant difference of global LV strain or strain rates between IIM patients and controls. Diffuse interstitial fibrosis can be detected by CMR T1 mapping in patients with IIM who do not have LGE or reduced LVEF or elevated hs-cTnI, and it may be a promising method for screening subclinical cardiac involvement in IIM. • Myocardial abnormality in IIM is often subclinical and leads to poor prognosis. • Conventional CMR parameters have limitations in early detection of cardiac function and tissue changes. • CMR T1 mapping techniques and myocardial strain analysis have the potential to provide detailed information on cardiac histology and function.

The aim of the study was to evaluate the role of cardiac magnetic resonance (CMR) mapping and strain analysis in the identification of cardiac involvement in idiopathic inflammatory myopathy (IIM) patients with preserved left ventricular ejection fraction. In all, 38 IIM patients who underwent CMR examination at our institution were retrospectively included. Twenty-three age-matched healthy individuals served as controls. Mapping parameters including native T1, extracellular volume (ECV), and T2 mapping and strain parameters including global radial strain, global circumferential strain, and global longitudinal strain were measured semiautomatically using a dedicated processing software. All the mapping and strain values were compared between patients and controls. Late gadolinium enhancement was only present in IIM patients (n=17, 44.7%). IIM patients showed higher native T1 (1346 vs. 1269 ms, P<0.001), ECV (31.1% vs. 27.4%, P<0.01), and higher T2 (44.4 vs. 39.2 ms, P<0.001) values compared with controls. The global radial strain (36.7% vs. 46.9%, P<0.001), global circumferential strain (-21.2% vs. -24.1%, P<0.01), and global longitudinal strain (-13.6% vs. -15.6%, P<0.05) values were significantly reduced compared with controls. Native T1, ECV, T2 values, and global strain values may hold promise for the detection of subclinical myocardial involvement in IIM patients with preserved left ventricular ejection fraction.

The diagnostic value of serum YKL-40 for myocardial involvement in idiopathic inflammatory myopathy

Myocardial fibrosis occurs in the early subclinical stage of cardiac involvement in idiopathic inflammatory myopathies (IIMs). Soluble suppression of tumorigenicity 2 (sST2) is known to have an immunomodulatory impact during autoimmune disease development. The current study investigated the diagnostic value of sST2 for myocardial fibrosis during early stage of cardiac involvement in IIM. A total of 44 IIM patients with normal heart function and 32 age- and gender-matched healthy controls (HCs) were enrolled. Serum sST2 levels were measured by ELISA and cardiac magnetic resonance (CMR) parameters for myocardial fibrosis [native T1, extracellular volume (ECV), late-gadolinium enhancement (LGE)] and oedema (T2 values) were analysed. IIM patients had significantly higher sST2 levels than HCs [67.5 ng/ml (s.d. 30.4)] vs 14.4 (5.5), P < 0.001] and levels correlated positively with diffuse myocardial fibrosis parameters, native T1 (r = 0.531, P = 0.000), ECV (r = 0.371, P = 0.013) and focal myocardial fibrosis index and LGE (r = 0.339, P = 0.024) by Spearman's correlation analysis. sST2 was an independent predictive factor for diffuse and focal myocardial fibrosis after adjustment for age, gender, BMI and ESR. Risk increased ≈15.4% for diffuse [odds ratio (OR) 1.154 (95% CI 1.021, 1.305), P = 0.022] and 3.8% for focal [OR 1.038 (95% CI 1.006, 1.072), P = 0.020] myocardial fibrosis per unit increase of sST2. Cut-off values for diagnosing diffuse and focal myocardial fibrosis were sST2 ≥51.3 ng/ml [area under the curve (AUC) = 0.942, sensitivity = 85.7%, specificity = 98.9%, P < 0.001] and 53.3 ng/ml (AUC = 0.753, sensitivity = 87.5%, specificity = 58.3%, P < 0.01), respectively. sST2 showed a marked elevation during the subclinical stage of cardiac involvement in IIM and has potential as a biomarker for predicting diffuse and focal myocardial fibrosis in IIM.

Cardiac involvement in idiopathic inflammatory myopathies (IIM) adversely affects prognosis but is commonly sub-clinical. Cardiac magnetic resonance imaging (CMR) is an effective imaging modality for detecting myocardial inflammation and fibrosis but its use as a screening tool for cardiac disease in IIM has not been fully explored. Nineteen patients with IIM without cardiac symptoms underwent CMR using a specific cardiomyopathy protocol including specific sequences detecting focal and diffuse myocardial fibrosis. 9/19 patients demonstrated late gadolinium enhancement (LGE (3/9 right ventricular insertion, 1/9 sub-endocardial, 7/9 mid-wall/sub-epicardial)). T1 mapping was performed in 15 patients. In total, 7/15 had elevated native T1 values, of which four had detected LGE. Myocardial fibrosis was frequently detected in IIM patients without cardiac history. Detection of LGE and elevated T1 values may have negative prognostic implications. Longitudinal studies determining whether early or augmented treatment has a role in patients with sub-clinical cardiac involvement are needed.Key Points• Cardiac involvement in myositis adversely affects prognosis.• Cardiac magnetic resonance imaging is an effective tool for detecting cardiac involvement.• T1 mapping is a technique which detects diffuse myocardial inflammation and fibrosis.• In our study, focal and diffuse myocardial fibrosis was frequently found in myositis patients without cardiac symptoms.

Cardiovascular involvement in idiopathic inflammatory myopathies (IIM) is an understudied area which is gaining increasing recognition in recent times. Recent advances in imaging modalities and biomarkers have allowed the detection of subclinical cardiovascular manifestations in IIM. However, despite the availability of these tools, the diagnostic challenges and underestimated prevalence of cardiovascular involvement in these patients remain significant. Notably, cardiovascular involvement remains one of the leading causes of mortality in patients with IIM. In this narrative literature review, we outline the prevalence and characteristics of cardiovascular involvement in IIM. Additionally, we explore investigational modalities for early detection of cardiovascular involvement, as well as newer approaches in screening to facilitate timely management.Key points• Cardiac involvement in IIM in majority cases is subclinical and a major cause of mortality.• Cardiac magnetic resonance imaging is sensitive for detection of subclinical cardiac involvement.

This review presents a detailed study of original researches and previously published reviews concerning cardiovascular involvement in idiopathic inflammatory myopathies (IIM). We aimed to summarize the current knowledge on the cardiac involvement in IIM, evaluate its impact on mortality and indicate areas still awaiting to be investigated. We searched MEDLINE database (until January 2019) and the reference lists of articles. Selection criteria included only published data, available in English, both original researches and reviews. Articles related to cardiovascular involvement in IIM were selected and analysed. The references were also screened, and relevant articles were included. Cardiovascular involvement is frequent in IIM but typically remains subclinical. Among far less prevalent symptomatic forms, congestive heart failure is the most common. Myocardium and conduction system seems to be predominantly affected. High rate of left ventricular diastolic dysfunction was observed. Non-specific changes of ST-T segment were the most common abnormalities in electrocardiography. Patients with IIM were more frequently affected by atrial fibrillation as compared with other autoimmune diseases. Increased risk of myocardial infarction was observed; furthermore, patients often develop comorbidities that enhance cardiovascular risk. Since cardiovascular disorders remain one of the major causes of death and subclinical involvement is frequent, active screening is justified. Growing availability of the novel imaging techniques may facilitate diagnosis. Correlation between myocardial involvement and the type of autoantibodies and impact of different therapeutic options on the progression of cardiovascular lesions require further studies.