Cardiac Involvement In Idiopathic Inflammatory Myopathies Research Articles

The diagnostic value of sST2 for myocardial fibrosis in idiopathic inflammatory myopathies in subclinical stage of cardiac involvement.

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.

Using multi-parametric quantitative MRI to screen for cardiac involvement in patients with idiopathic inflammatory myopathy

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.

Defining cardiac involvement in idiopathic inflammatory myopathies: a systematic review.

Recent advances in cardiac MRI (CMR) and other diagnostic techniques have made it easier to identify subclinical cardiac inflammation and dysfunction in the idiopathic inflammatory myopathies (IIM). Herein, we systematically review the literature regarding cardiac involvement in IIM. We searched Medline and EMBASE from 1990 to 2020 using keywords related to IIM and cardiac disease. We included English language studies in adults with any immune-mediated, inflammatory muscle pathology. We identified 10 425 potentially relevant abstracts, of which 29 were included. Most frequently these included patients with PM or DM without symptomatic myocarditis. Five categories of cardiac investigation were used in these patients: cardiac enzyme testing, ECG, transthoracic echocardiography, CMR and nuclear medicine testing. Patients with clinical myocarditis had universally abnormal cardiac troponin levels and ECG. Elevated cardiac troponin T was more common than cardiac troponin I and may correlate with disease activity, whereas cardiac troponin I was more specific for cardiac involvement. Non-specific ECG changes were common. The major finding on transthoracic echocardiography was abnormal ejection fraction. Gross systolic dysfunction was unusual, but subclinical systolic dysfunction was reported in several studies. Abnormal diastolic function was common and may be associated with disease duration. Late gadolinium enhancement (reflecting regional necrosis or scarring) and abnormal myocardial mapping parameters (reflecting myocardial inflammation, fibrosis and oedema) were frequently identified on CMR, suggesting significant subclinical myocardial pathology (despite typically normal ejection fraction). Abnormal cardiac investigations are commonly found in asymptomatic IIM patients, which has potential prognostic and treatment implications.

Muscle pathological features and extra-muscle involvement in idiopathic inflammatory myopathies with anti-mitochondrial antibody

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.

Necrotizing Myopathy Resulting In Myocarditis And Sudden Death

AbstractNecrotizing myopathy (NM), a type of idiopathic inflammatory myopathy (IIM), is an autoimmune disorder in which immune cells target skeletal muscle, resulting in muscle breakdown and weakness. Cardiac involvement in this disorder is rare. We highlight a case of autoantibody-negative NM complicated by myocarditis and sudden cardiac death.Case Report: A 45-year-old male with a history of hypertension, previous hepatitis B, and obesity presented with 6 months of progressive proximal lower extremity weakness. Workup revealed elevated creatinine phosphokinase, ESR and CRP, elevated troponin, and electromyography notable for a myopathic process. Age appropriate malignancy screening, infectious workup, and myositis panel were negative. The patient underwent left bicep biopsy which revealed muscle fiber necrosis and regeneration, consistent with the diagnosis of seronegative NM. Patient was discharged on mycophenolate and intravenous immunoglobulin with improvements in peripheral strength. Unfortunately, while recovering, he was readmitted with palpitations and dyspnea. Electrocardiogram showed a new trifascicular block and telemetry revealed frequent runs of non-sustained ventricular tachycardia (NSVT). A Cardiac MRI revealed evidence of myocarditis (Image 1). Endomyocardial biopsy showed fibrosis with entrapped myocytes (Image 2). He was treated with pulse dose intravenous glucocorticoids and mycophenolate was increased. EP study (EPS) was performed which did not reveal inducible VT; therefore, implantable cardiac defibrillator (ICD) was not implanted. Three months later the patient had sudden unexplained death at home.Discussion: Cardiac involvement in IIM—especially seronegative NM—is rare and not well described. Our patient had worsening cardiac involvement despite improvements in his skeletal myopathy. Despite a recent negative EPS, the patient had sudden unexplained death. Perhaps, a lower threshold for ICD implantation should be considered in this population. This case raises awareness for cardiac involvement in seronegative NM and raises the question of whether these patients are at increased risk of sudden cardiac death.

CMR T1 mapping and strain analysis in idiopathic inflammatory myopathy: evaluation in patients with negative late gadolinium enhancement and preserved ejection fraction.

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.

Cardiovascular complications in patients with idiopathic inflammatory myopathies: does heart matter in idiopathic inflammatory myopathies?

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.

Using serum troponins to screen for cardiac involvement and assess disease activity in the idiopathic inflammatory myopathies.

ObjectivesLimitations in the methods available for identifying cardiac involvement and accurately quantifying disease activity in the idiopathic inflammatory myopathies (IIMs) may contribute to poor outcomes. We investigated the utility of different serum muscle damage markers [total creatine kinase (CK), cardiac troponin T (cTnT) and cardiac troponin I (cTnI)] to address these issues. MethodsWe assessed disease activity and cardiac involvement using the International Myositis Assessment and Clinical Studies Group core set measures in 123 participants with confirmed adult-onset IIM from the UK and Denmark. Total CK, cTnT and cTnI were measured. Associations were assessed using logistic regression and Spearman’s ranked correlation.ResultsCardiac involvement (n = 18) was associated with higher cTnI levels, independent of overall disease activity [adjusted odds ratio 1.03 (95% CI 1.01, 1.05); P = 0.002]. An abnormal cTnI had the highest specificity and positive predictive value for cardiac involvement (95% and 62%, respectively). In those with a normal CK but elevated cTnT or cTnI, an association with increased disease activity scores was observed. Serum cTnT correlated with the physician (ρ = 0.39) and patient-assessed (ρ = 0.28) global visual analogue scales and HAQ (ρ = 0.41) more strongly than CK or cTnI levels. cTnT was the only marker to correlate with manual muscle testing scores (ρ = −0.24).ConclusionSerum cTnI testing may have a role in screening for cardiac involvement in IIMs. Abnormal levels of serum cTnT and cTnI are associated with increased disease activity, including in those with a normal CK.

Cardiovascular involvement in myositis.

The purpose of this review is to provide an update on cardiovascular involvement in idiopathic inflammatory myopathy (IIM). Studies from the past 18 months are identified and reviewed. Finally, the clinical impact of these findings is discussed. Epidemiological studies have revealed an increased risk of myocardial infraction and venous thromboembolism (VTE) - including deep venous thrombosis and pulmonary embolism - in adults with polymyositis or dermatomyositis compared to the general population, even after adjustment for potential confounders. This increased risk applies particularly within the initial year of diagnosis. In addition, cross-sectional studies have shown subclinical cardiac involvement in IIM effecting both heart function and rhythm, and conduction abnormalities, which in part might be because of myocarditis. The International Consensus Group on cardiac magnetic resonance (CMR) imaging suggests that CMR should be considered as a potentially viable diagnostic tool to evaluate the possibility of silent myocardial inflammation in IIM with normal routine noninvasive evaluation. Updated literature on cardiovascular involvement in IIM has identified an increased risk for subclinical and clinical cardiovascular disease in these rare inflammatory muscle diseases.